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Relevant bibliographies by topics / Amino acids Metabolism Disorders

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Academic literature on the topic 'Amino acids Metabolism Disorders'

Author: Grafiati

Published: 4 June 2021

Last updated: 28 January 2023

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Journal articles on the topic "Amino acids Metabolism Disorders"

1

Litvitskii, P. F., and L. D. Mal'tseva. "PROTEIN, AMINO ACIDS AND NUCLEIC ACIDS METABOLISM DISORDERS." Current pediatrics 14, no. 1 (2015): 95–107. http://dx.doi.org/10.15690/vsp.v14i1.1267.

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2

Knerr, Ina, Laurie Bernstein, Ellen Crushell, Siobhan O’Sullivan, and Jörn Oliver Sass. "Amino Acids and Inherited Amino Acid-Related Disorders." Journal of Nutrition and Metabolism 2018 (September 10, 2018): 1–2. http://dx.doi.org/10.1155/2018/5629454.

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3

Owayes Muaffaq Hamed, Amjad Abdul-hadi Mohammed, and Raed Salem Alsaffar. "Genetic Metabolism Disorders in Newborn." International Journal for Research in Applied Sciences and Biotechnology 8, no. 1 (January 13, 2021): 77–81. http://dx.doi.org/10.31033/ijrasb.8.1.9.

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Babies with any type of metabolic disorders lack the ability to break down the food well, which may induce too little amino acids, phenylalanine and blood sugar to the body, there are numerous kinds of this disorders, most of babies with a genetic metabolic disease have many mutation in gene that coded an enzyme which results a deficiency in same enzyme are hundreds of these disorders and they were diagnosed by their symptoms and the treatment method. The treatment methods of the metabolic disorder depend on the specific type of disorders, inborn metabolic disease are some-time treated with dietary guidance, and other childcare choices, many hereditary metabolic disease are initially caused by gene mutations and that transferred from parents to offspring.

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Xie, Fei, Zhengqun Liu, Ming Liu, Liang Chen, Wei Ding, and Hongfu Zhang. "Amino Acids Regulate Glycolipid Metabolism and Alter Intestinal Microbial Composition." Current Protein & Peptide Science 21, no. 8 (November 9, 2020): 761–65. http://dx.doi.org/10.2174/1389203721666200219100216.

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Amino acids (AAs) and their metabolites regulate key metabolic pathways that are necessary for growth, reproduction, immunity and metabolism of the body. It has been convinced that metabolic diseases are closely related to disorders of glycolipid metabolism. A growing number of studies have shown that AAs are closely related to energy metabolism. This review focuses on the effects of amino acids (arginine, branched-chain amino acids, glutamine) and their metabolites (short chain fatty acids) on glycolipid metabolism by regulating PI3K/AKT/mTOR and AMPK signaling pathways and GPCRs receptors, reducing intestinal Firmicutes/Bacteroidetes ratio associated with obesity.

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Barvinska, O. I., N. V. Olkhovych, and N. G. Gorovenko. "Determination of the reference values of amino acids and acylcarnitines level in the newborn dry blood spots in Ukraine." Visnik ukrains'kogo tovaristva genetikiv i selekcioneriv 16, no. 1 (September 7, 2018): 12–19. http://dx.doi.org/10.7124/visnyk.utgis.16.1.898.

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Aim. Implementation of neonatal screening of the group of severe inherited disorders of amino acids, fatty and organic acids metabolism in Ukraine involves determination of the biological variation of amino acids and acylcarnitines level in the blood that depends on the gender, geographical origin of the samples and the gestation term of newborn. Methods. The main method of rapid and reliable diagnosis of this group of inherited disorders is determination of amino acids and acylcarnitines level by liquid chromatography tandem mass spectrometry. Results. Reference intervals of amino acids and acylcarnitines concentration in dry blood samples of newborns from different regions of Ukraine haven’t differed. However, it was found necessity to use separate reference intervals of some amino acids and acylcarnitines (citrulline, arginine, methionine, leucine, tyrosine, alanine, histidine, C0, C3, C3DC, C4, C5, C6, C8, C8:1, C10:2, C12, C14:1, C16, C18OH, C18:1OH) for premature and full-term newborns. Also, it was revealed that for male and female newborns it is necessary to use different reference intervals of citrulline, leucine, C8:1, arginine, methionine, C5, C8. Conclusions. In this study it was found that variation of amino acids and acylcarnitines concentration in dry blood spots depends on the newborns gestation term and sex. We have proposed a two-stage procedure of biomarkers interpretation for which the values variation in different neonatal groups was detected. Keywords: amino acids and acylcarnitines reference intervals, inherited disorders of amino acids, fatty and organic acids metabolism, newborn screening.

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Sperl, W., D. Skladal, W. Endres, G. Speer, and K. Groke. "Parenteral administration of amino acids in disorders of branched-chain amino acid metabolism." Journal of Inherited Metabolic Disease 17, no. 6 (1994): 753–54. http://dx.doi.org/10.1007/bf00712021.

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7

Cheng, Yu-Jung, Chieh-Hsin Lin, and Hsien-Yuan Lane. "d-Amino Acids and pLG72 in Alzheimer’s Disease and Schizophrenia." International Journal of Molecular Sciences 22, no. 20 (October 9, 2021): 10917. http://dx.doi.org/10.3390/ijms222010917.

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Numerous studies over the last several years have shown that d-amino acids, especially d-serine, have been related to brain and neurological disorders. Acknowledged neurological functions of d-amino acids include neurotransmission and learning and memory functions through modulating N-methyl-d-aspartate type glutamate receptors (NMDARs). Aberrant d-amino acids level and polymorphisms of genes related to d-amino acids metabolism are associated with neurodegenerative brain conditions. This review summarizes the roles of d-amino acids and pLG72, also known as d-amino acid oxidase activator, on two neurodegenerative disorders, schizophrenia and Alzheimer’s disease (AD). The scope includes the changes in d-amino acids levels, gene polymorphisms of G72 genomics, and the role of pLG72 on NMDARs and mitochondria in schizophrenia and AD. The clinical diagnostic value of d-amino acids and pLG72 and the therapeutic importance are also reviewed.

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Galan, I. O., S. T. Omelychuk, R. G. Protsyuk, V. I. Petrenko, T. I. Anistratenko, and A. V. Galan. "PREBIOTICS AND NUTRITIONAL CORRECTION IN INTEGRATED THERAPY OF PATIENTS WITH PULMONARY TUBERCULOSIS AND COMORBID PROTEIN METABOLIC DISORDERS." Актуальні проблеми сучасної медицини: Вісник Української медичної стоматологічної академії 20, no. 2 (July 6, 2020): 23–30. http://dx.doi.org/10.31718/2077-1096.20.2.23.

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The purpose of the work is to study the effectiveness of prebiotics and nutritional correction as components of the integrated therapy of patients with pulmonary tuberculosis and protein metabolic disorders. 67 patients with pulmonary tuberculosis were examined and then divided into two groups: I group included 35 patients, who received standard antimycobacterial therapy in the intensive phase of the treatment in combination with prebiotic (lactulose) in a dose of 20 ml 3 times a day plus nutriological correction of metabolic disorders; the II group included 32 patients, who received standard antimycobacterial therapy. The control group consisted of 30 healthy individuals of comparable sex and age. In order to study the protein metabolism state, the content of certain substitutable amino acids (ornithine, aspartic acid, serine, glutamic acid, proline, glycine, alanine, cysteine, tyrosine, glutamine) and essential amino acids (lysine, histidine, arginine, threonine, valine, methionine, isoleucine, phenylalanine, leucine) and their total amount (mg per 100 ml of blood serum) were assessed. Results and discussion. It has been found that all patients in groups I and II, which were under our supervision before the treatment, had symptoms of intoxication and respiratory syndrome, the tuberculosis process in the lungs occupied more than three segments and single decaying and rupturing cavities prevailed. The patients with massive bacterial excretion dominated in both groups. Moreover, before the treatment, protein metabolic disorders were found out in both groups. They manifested by the imbalance of individual amino acids and led to a decrease in the total content of essential amino acids in 1,2 – 1,3 times, total content of substitutable amino acids in 1, 2 times that led to a decrease in total content of substitutable amino acids in patients of both groups in 1,2 – 1,3 in comparison with the control group. A comparative analysis of the effectiveness of prebiotics (lactulose) and nutritional correction of metabolic disorders in combination with antimycobacterial therapy in the TB patientsl with protein metabolic disorders showed that probiotics and dietary modifications contribute to improving protein metabolism. This has been evidenced by a significantly high (p <0,05) level of the total content of essential amino acids in blood serum, total content of substitutable amino acids and total content of amino acids (reliably reaching the level of indicators in healthy individuals) compared with the relevant indicators in the group of the patients receiving only antimycobacterial therapy. Probiotics and nutritional correction promote the rapid dynamics of the disappearance of respiratory syndromes and intoxication, promote cavity healing, and reduce the duration of treatment. Conclusions. The results of our research obtained convincingly suggest that prior to the beginning of treatment; all patients with pulmonary tuberculosis have protein metabolic disorders that require selecting a way to correct these disorders in order to increase the effectiveness of TB treatment. Antimycobacterial therapy during two months contributes to the positive dynamics of protein metabolism correction, but complete normalization of the amino acid composition in blood serum does not occur. Applying prebiotics (lactulose) and nutriological correction of metabolic disorders in combination with antimycobacterial therapy promotes the normalization of protein metabolism and increases the effectiveness of the TB treatment.

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Medghalchi, Abdolreza. "The Effect of Amino Acid, Carbohydrate, and Lipid Metabolism Disorders on Eyes." Caspian Journal of Neurological Sciences 6, no. 3 (July 1, 2020): 190–96. http://dx.doi.org/10.32598/cjns.6.22.5.

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Inherited metabolic disorders (IMDs) are a class of genetic disorders. Each metabolic disorder may have different forms with different age of onset, clinical manifestations, severity, and even type of inheritance. Ideally, a group of different specialists, including ophthalmologists, pediatricians, biochemists, and medical geneticists are needed for the final diagnosis and management of IMDs. Because of the importance of the aforementioned issue, we investigated the effect of IMDs on the eye in this review. Metabolic disorders can induce abnormalities in conjunctiva, cornea, lens, retina, optic nerve, and eye motility. In this study, the authors aimed to address the effect of metabolic diseases of amino acids, carbohydrates, and lipids on eye metabolism. Because of the direct toxic mechanisms of abnormal metabolites on the eyes and regarding the effect of eye monitoring on follow-up, management, and treatment of IMDs, a detailed ophthalmological assessment is essential.

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Kožich, Viktor, and Sally Stabler. "Lessons Learned from Inherited Metabolic Disorders of Sulfur-Containing Amino Acids Metabolism." Journal of Nutrition 150, Supplement_1 (October 1, 2020): 2506S—2517S. http://dx.doi.org/10.1093/jn/nxaa134.

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ABSTRACT The metabolism of sulfur-containing amino acids (SAAs) requires an orchestrated interplay among several dozen enzymes and transporters, and an adequate dietary intake of methionine (Met), cysteine (Cys), and B vitamins. Known human genetic disorders are due to defects in Met demethylation, homocysteine (Hcy) remethylation, or cobalamin and folate metabolism, in Hcy transsulfuration, and Cys and hydrogen sulfide (H2S) catabolism. These disorders may manifest between the newborn period and late adulthood by a combination of neuropsychiatric abnormalities, thromboembolism, megaloblastic anemia, hepatopathy, myopathy, and bone and connective tissue abnormalities. Biochemical features include metabolite deficiencies (e.g. Met, S-adenosylmethionine (AdoMet), intermediates in 1-carbon metabolism, Cys, or glutathione) and/or their accumulation (e.g. S-adenosylhomocysteine, Hcy, H2S, or sulfite). Treatment should be started as early as possible and may include a low-protein/low-Met diet with Cys-enriched amino acid supplements, pharmacological doses of B vitamins, betaine to stimulate Hcy remethylation, the provision of N-acetylcysteine or AdoMet, or experimental approaches such as liver transplantation or enzyme replacement therapy. In several disorders, patients are exposed to long-term markedly elevated Met concentrations. Although these conditions may inform on Met toxicity, interpretation is difficult due to the presence of additional metabolic changes. Two disorders seem to exhibit Met-associated toxicity in the brain. An increased risk of demyelination in patients with Met adenosyltransferase I/III (MATI/III) deficiency due to biallelic mutations in the MATIA gene has been attributed to very high blood Met concentrations (typically >800 μmol/L) and possibly also to decreased liver AdoMet synthesis. An excessively high Met concentration in some patients with cystathionine β-synthase deficiency has been associated with encephalopathy and brain edema, and direct toxicity of Met has been postulated. In summary, studies in patients with various disorders of SAA metabolism showed complex metabolic changes with distant cellular consequences, most of which are not attributable to direct Met toxicity.

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Dissertations / Theses on the topic "Amino acids Metabolism Disorders"

1

Smith, Douglas W. 1961. "The lysinuric protein intolerance phenotype : amino acid transport in cultured skin fibroblasts." Thesis, McGill University, 1986. http://digitool.Library.McGill.CA:80/R/?func=dbin-jump-full&object_id=65416.

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Fu, Katherine. "Isolation of human BCAD gene and analysis of putative BCAD deficiency." Thesis, McGill University, 1993. http://digitool.Library.McGill.CA:80/R/?func=dbin-jump-full&object_id=68175.

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The 2-methylbranched chain acyl-CoA dehydrogenase (BCAD) is a mitochondrial enzyme that catalyzes the third reaction in isoleucine and valine metabolism, the oxidation of 2-methylbutyryl-CoA and isobutyryl-CoA, respectively. BCAD deficiency would result in the accumulation of branched chain acyl-CoAs or their derivatives. Three patients with a putative defect in BCAD have been reported. This study consists of a molecular examination of one such patient as well as the characterization of the BCAD gene. In Northern blot analysis of human fibroblast RNA, the BCAD cDNA hybridized to two RNA species of 2.7 and 6.5 kb. The 2.7 kb band corresponds to the size of the BCAD cDNA, which consists of the entire coding region of 1.3 kb and a 3$ sp prime$ untranslated region of 1.4 kb. The coding regions of the BCAD gene span approximately 21 kb and consist of 12 exons and 11 introns. The exons range in size from 39 to 108 bp. In the analysis of the putative BCAD-deficient patient, no significant difference was observed at the level of DNA (Southern), RNA (Northern) or protein (Western) when compared to controls, suggesting that the BCAD gene in this patient did not contain any large insertions or deletions, or a frameshift mutation. The single strand conformation polymorphism (SSCP) technique and sequencing of the entire coding region did not reveal any disease-causing mutations but two polymorphisms were identified: one in exon 6 and the other in exon 10.

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Campeau, Eric. "Molecular genetics of biotin-dependent enzymes : mutation analysis, expression and biochemical studies." Thesis, National Library of Canada = Bibliothèque nationale du Canada, 1999. http://www.collectionscanada.ca/obj/s4/f2/dsk1/tape7/PQDD_0019/NQ55308.pdf.

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Dumas, Richard. "The intracellular localization of holocarboxylase synthetase." Thesis, National Library of Canada = Bibliothèque nationale du Canada, 1999. http://www.collectionscanada.ca/obj/s4/f2/dsk1/tape7/PQDD_0018/MQ55050.pdf.

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Dupuis, Lucie. "Molecular basis of biotin-responsive multiple carboxylase deficiency." Thesis, McGill University, 1996. http://digitool.Library.McGill.CA:80/R/?func=dbin-jump-full&object_id=27309.

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Multiple carboxylase deficiency (MCD) results from a decreased activity of holocarboxylase synthetase (HCS) which is responsible for the biotinylation of the four biotin-dependent carboxylases found in humans. The disease can be treated with pharmacologic doses of oral biotin (biotin-responsiveness). The cDNA for HCS contains a biotin-binding domain deduced by analogy with the sequence and crystal structure of the E. coli BirA biotin ligase. E. coli birA$ sp-$ mutations causing biotin-auxotrophy all localize to this region. Of six point mutations I have identified in MCD patients, four localize to the biotin-binding region. In order to assess the HCS activity associated with patient mutations, I used an assay based on the expression of mutant HCS in E. coli. The method is based on the ability of mutant HCS to biotinylate the biotin carboxyl carrier protein (BCCP) of acetyl-CoA carboxylase in a temperature-sensitive birA$ sp-$ E. coli strain using 3H-biotin as tracer. I have shown that all of the mutations cause a severe decrease in HCS activity. In addition, I have shown that five of the mutant HCS are biotin-responsive. These findings are a major contribution to the understanding of the mechanism of biotin-responsiveness.

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Léon, Del Rio Alfonso. "Molecular genetics of holocarboxylase synthetase deficiency." Thesis, McGill University, 1995. http://digitool.Library.McGill.CA:80/R/?func=dbin-jump-full&object_id=29074.

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The objective of this thesis was to determine the molecular basis of neonatal multiple carboxylase deficiency (MCD) produced by an impairment in holocarboxylase synthetase (HCS) activity and the origin of the biotin-responsiveness that characterizes this disease. To determine HCS activity, I developed a peptide substrate and used the biotinylation system of E: coli to determine its properties. C-terminal fragments of the $ alpha$ subunit of human propionyl-CoA carboxylase (PCC-$ alpha$) were expressed in E. coli and site-directed mutagenesis was used to define the residues required for biotinylation by the bacterial biotin ligase, BirA. These experiments showed that the biotin region of PCC-$ alpha$ can act as an autonomous domain for biotinylation and suggested its use as substrate for human HCS. For the molecular characterization of MCD, I isolated several cDNA clones encoding human HCS by functional complementation of an E. coli mutant with a temperature-sensitive BirA. Comparison of the predicted amino acid sequence of HCS with bacterial biotin ligases allowed the identification of the putative biotin-binding domain of this protein. Mutation analysis of DNA from HCS deficient patients showed that most of the changes in the HCS sequence are clustered in the biotin-binding domain. All the patients tested in this study showed deficiency of HCS activity as determined using the PCC-$ alpha$ peptide as substrate for biotinylation. The biotin-responsiveness was demonstrated by obtaining a stimulation of HCS activity of MCD cells at high biotin concentrations while remaining unstimulated in extracts of normal cells. Together with the mutation studies, these results showed that neonatal MCD is caused by mutations in the biotin binding domain of HCS which reduce the affinity of the enzyme towards biotin. This change in the kinetic properties of HCS results in the inefficient biotinylation of carboxylases at physiological concentrations of biotin. The defect can be over

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Vicanek, Caroline Michaela. "Expression studies on the shortbranched chain acyl-CoA dehydrogenase (SBCAD) gene." Thesis, McGill University, 1995. http://digitool.Library.McGill.CA:80/R/?func=dbin-jump-full&object_id=22824.

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Short/branched chain acyl-CoA dehydrogenase (SBCAD), a member of the acyl-CoA dehydrogenase (ACD) family of enzymes, catalyzes the oxidation of branched chain fatty acids and the branched chain amino acids isoleucine and valine. This research project focuses on expression studies of the SBCAD gene. Northern blot analysis detected two SBCAD mRNA species of 2.7 and 6.5 kb in various human tissues and cell types. A single 4.1 and 2.0 kb SBCAD message was detected in rat and pig tissues, respectively, revealing a species difference in SBCAD mRNA size. Studies of human and rat SBCAD tissue-specificity and relative abundance, at both the RNA and protein levels, identified liver and kidney as the tissues with the highest levels of SBCAD expression, establishing a unique tissue-specific expression pattern that is not seen among the other members of the ACD family. Furthermore, a fetal and adult difference in SBCAD expression was observed in human kidney, suggesting that the SBCAD gene may be developmentally regulated in some tissues. Finally, an attempt was made to isolate and characterize the SBCAD promoter region in order to provide valuable data for future SBCAD promoter studies.

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Hamadeh, Mazen Jamal. "Methods for detecting abnormal adaptation to protein restriction in humans with special reference to insulin-dependent diabetes mellitus." Thesis, McGill University, 2001. http://digitool.Library.McGill.CA:80/R/?func=dbin-jump-full&object_id=36948.

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Postprandial urea production in subjects with insulin dependent diabetes mellitus (IDDM) on conventional insulin therapy is normal when the previous diet is high in protein, but there is an incomplete adaptive reduction in urea production following protein restriction. To evaluate the nutritional implications of restricted protein intake in human diabetes mellitus, it is first necessary to establish a reliable method to measure changes in urea production and amino acid catabolism in response to changes in dietary protein intake. We therefore tested (1) the accuracy of the urea production rate (Ra) to depict changes in urea production, (2) whether sulfate production can be accurately depicted using tracer or nontracer approaches, after establishing the use of electrospray tandem mass spectrometry to measure sulfate concentrations and 34SO4 enrichments following administration of the stable isotope tracer sodium [34S]sulfate, (3) the reproducibility of urea and sulfate measurements following a test meal low in protein (0.25 g/kg) in subjects previously adapted to high (1.5 g/kg.d) and low (0.3 g/kg.d) protein intakes, and compared the metabolic fate of [ 15N]alanine added to the test meal with that of [15N] Spirulina platensis, a 15N-labeled intact protein, and (4) whether we could identify the differences in postprandial urea and sulfate productions between normal subjects and persons with IDDM receiving conventional insulin therapy previously adapted to high protein intake, when the test meal was limiting in protein. Under basal conditions, steady state urea Ra is an accurate measure of urea production. Following changes in urea production, both the tracer and nontracer methods seriously underestimated total urea Ra. The tracer method overestimated sulfate production by 20%, but the nontracer method provided an accurate measure of sulfate production and, hence, sulfur amino acid catabolism. Postprandial changes in urea and sulfate productions following normal ada

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Miller, John H. IV. "A NEW APPROACH TO DRIED BLOOD SPOT ANALYSIS FOR NEWBORN SCREENING USING HIGH RESOLUTION LIQUID CHROMATOGRAPHY TANDEM MASS SPECTROMETRY." VCU Scholars Compass, 2012. http://scholarscompass.vcu.edu/etd/2906.

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The primary purpose of newborn screening is to quickly identify children that are at risk of having a specific disorder in order to start treatment, prevent early death and reduce the chances of permanent physical or mental damage. The current and widely accepted approach used for identification of metabolism disorders involves a flow injection analysis with mass spectrometry detection of acylcarnitines and amino acids. Although this approach is widely accepted and has shown to be sufficient for identification of multiple metabolism disorders the method is not fully quantitative and results often have to be confirmed by second-tier tests. The primary focus of this research was to improve the accuracy and selectivity of this screening method by employing a high resolution chromatographic separation for the combined analysis of twelve acylcarnitines and seven amino acids. This method is an improvement over the current methodology allowing for separation of key isomers that are diagnostic for different metabolism disorders, reducing the need for multiple second-tier tests to confirm results and shortening the time to diagnosis. In order to further improve the efficiency of newborn screening we developed an in-line desorption device, which allows for direct analysis of DBS eliminating the need for punching disks from the filter paper cards. Our device was the first published paper that demonstrated the ability to directly analyze dried blood spots, without the need for any offline sample processing. Using this device, we validated a method to quantify biomarkers related to Maple Syrup Urine Disease, a disorder that requires a second-tier test for confirmation. To further improve the accuracy of dried blood spot analysis we evaluated a technique to correct the sample volume in low and high hematocrit samples. The level of hematocrit in blood spotted on filter paper cards affects the volume of sample analyzed, leading to errors in accuracy. Diffuse reflectance was used to relate differences in sample hematocrit on dried blood spots. We validated our technique with eighteen donor samples at various levels of hematocrit. Correcting sample volume for hematocrit showed improved precision and accuracy over the standard approach, ultimately reducing the potential to misidentify samples.

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Showiheen, Salah Ali A. "Metabolomics profiling of amino acids metabolism in osteoarthritis." Thesis, Queensland University of Technology, 2018. https://eprints.qut.edu.au/123249/1/Salah%20Ali%20A_Showiheen_Thesis.pdf.

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The researcher studied the role of amino acid metabolism in osteoarthritis progression. The study suggests that this abnormal amino acid metabolism aids in the development of the disease. This data further suggests that amino acids could be potential circulatory markers for diagnosing OA and therapeutic strategies of amino acids supplementation could be considered as a potential treatment.

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Books on the topic "Amino acids Metabolism Disorders"

1

Kollegger, Harald. Excitatory amino acids and brain damage. Wien: Facultas-Universitätsverlag, 1993.

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Pisters, Peter W. T., 1960- and Brennan Murray F, eds. Protein and amino acid metabolism in cancer cachexia. New York: Chapman & Hall, 1996.

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1918-, Bickel Horst, and Wachtel U, eds. Inherited diseases of amino acid metabolism: Recent progress in the understanding, recognition, and management : international symposium in Heidelberg 1984. Stuttgart ; New York: Thieme, 1985.

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M, Ostrovskiĭ I͡U. Aminokisloty v patogeneze, diagnostike i lechenii alkogolizma. Minsk: Navuka i tėkhnika, 1995.

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Jones, Patricia M. Quick guide to organic acid interpretation. Washington D.C: AACC Press, 2011.

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Toshihiko, Andō, ed. Aminosan to seikatsu shūkanbyō: Saishin aminoguramu de saguru "inochi" no kagaku = Amino acids. Tōkyō: Joshi Eiyō Daigaku Shuppanbu, 2010.

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Parker, James N., and Philip M. Parker. Maple syrup urine disease: A bibliography and dictionary for physicians, patients, and genome researchers [to Internet references]. San Diego, CA: ICON Health Publications, 2007.

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Parker, James N., and Philip M. Parker. Beta-ketothiolase deficiency: A bibliography and dictionary for physicians, patients, and genome researchers [to Internet references]. San Diego, CA: ICON Health Publications, 2007.

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Parker, James N., and Philip M. Parker. 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: A bibliography and dictionary for physicians, patients, and genome researchers [to internet references]. San Diego, CA: ICON Health Publications, 2007.

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Bender, David A. Amino acid metabolism. 3rd ed. Chichester, West Sussex: John Wiley & Sons, 2012.

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Book chapters on the topic "Amino acids Metabolism Disorders"

1

Braun-Falco, Otto, Gerd Plewig, Helmut H. Wolff, and Walter H. C. Burgdorf. "Disorders of Amino Acid Metabolism." In Dermatology, 1261–68. Berlin, Heidelberg: Springer Berlin Heidelberg, 2000. http://dx.doi.org/10.1007/978-3-642-97931-6_39.

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Vermesh, Michael, and Norbert Gleicher. "Disorders of Amino Acid Metabolism." In Principles of Medical Therapy in Pregnancy, 283–85. Boston, MA: Springer US, 1985. http://dx.doi.org/10.1007/978-1-4613-2415-7_26.

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Skovby, Flemming. "Disorders of Sulfur Amino Acids." In Physician’s Guide to the Laboratory Diagnosis of Metabolic Diseases, 243–60. Berlin, Heidelberg: Springer Berlin Heidelberg, 2003. http://dx.doi.org/10.1007/978-3-642-55878-8_16.

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Arnold, Georgianne L., and Jerry Vockley. "Disorders of Metabolism of Amino Acids and Related Compounds." In Genetic Disorders and the Fetus, 877–902. Hoboken, NJ, USA: John Wiley & Sons, Inc., 2015. http://dx.doi.org/10.1002/9781118981559.ch23.

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Kožich, Viktor, Andrew A. M. Morris, and Henk J. Blom. "Disorders of Sulfur Amino Acid Metabolism." In Inborn Metabolic Diseases, 309–20. Berlin, Heidelberg: Springer Berlin Heidelberg, 2016. http://dx.doi.org/10.1007/978-3-662-49771-5_20.

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Andria, Generoso, Brian Fowler, and Gianfranco Sebastio. "Disorders of Sulfur Amino Acid Metabolism." In Inborn Metabolic Diseases, 224–31. Berlin, Heidelberg: Springer Berlin Heidelberg, 2000. http://dx.doi.org/10.1007/978-3-662-04285-4_18.

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Yudkoff, M. "13 Disorders of Amino Acid Metabolism." In Handbook of Neurochemistry and Molecular Neurobiology, 277–303. Boston, MA: Springer US, 2007. http://dx.doi.org/10.1007/978-0-387-30373-4_13.

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Andria, Generoso, Brian Fowler, and Gianfranco Sebastio. "Disorders of Sulfur Amino Acid Metabolism." In Inborn Metabolic Diseases, 311–21. Berlin, Heidelberg: Springer Berlin Heidelberg, 2012. http://dx.doi.org/10.1007/978-3-642-15720-2_21.

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Kožich, Viktor, Andrew A. M. Morris, and Henk J. Blom. "Disorders of Sulfur Amino Acid Metabolism." In Inborn Metabolic Diseases, 407–22. Berlin, Heidelberg: Springer Berlin Heidelberg, 2022. http://dx.doi.org/10.1007/978-3-662-63123-2_20.

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Andria, Generoso, Brian Fowler, and Gianfranco Sebastio. "Disorders of Sulfur Amino Acid Metabolism." In Inborn Metabolic Diseases, 273–82. Berlin, Heidelberg: Springer Berlin Heidelberg, 2006. http://dx.doi.org/10.1007/978-3-540-28785-8_21.

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Conference papers on the topic "Amino acids Metabolism Disorders"

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Kidd, Catherine, Rachel Skeath, and Marjorie Dixon. "55 Improving cost-effective prescribing of low-protein foods for dietary management for disorders of amino acid (AA) metabolism." In GOSH Conference 2019, Care of the Complex Child. BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health, 2019. http://dx.doi.org/10.1136/archdischild-2019-gosh.55.

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Nicol, T., S. Podliesna, V. Portero, S. Falcone, A. Blease, S. Casini, CR Bezzina, CA Remme, and P. Potter. "T3 Cardiac arrhythmia resulting from an accumulation of branched chain amino acids in a mouse line with a mutation in bcat2." In British Society for Cardiovascular Research, Autumn Meeting 2017 ‘Cardiac Metabolic Disorders and Mitochondrial Dysfunction’, 11–12 September 2017, University of Oxford. BMJ Publishing Group Ltd and British Cardiovascular Society, 2018. http://dx.doi.org/10.1136/heartjnl-2018-bscr.3.

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Krželj, Vjekoslav, and Ivana Čulo Čagalj. "INHERITED METABOLIC DISORDERS AND HEART DISEASES." In Symposium with International Participation HEART AND … Akademija nauka i umjetnosti Bosne i Hercegovine, 2019. http://dx.doi.org/10.5644/pi2019.181.02.

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Inherited metabolic disorders can cause heart diseases, cardiomyopathy in particular, as well as cardiac arrhythmias, valvular and coronary diseases. More than 40 different inherited metabolic disorders can provoke cardiomyopathy, including lysosomal storage disorders, fatty acid oxidation defects, organic acidemias, amino acidopathies, glycogen storage diseases, congenital disorders of glycosylation as well as peroxisomal and mitochondrial disorders. If identified and diagnosed on time, some of congenital metabolic diseases could be successfully treated. It is important to assume them in cases when heart diseases are etiologically undefined. Rapid technological development has made it easier to establish the diagnosis of these diseases. This article will focus on common inherited metabolic disorders that cause heart diseases, as well as on diseases that might be possible to treat.

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Resink, J. W., and T. A. T. G. van Kempen. "Protective effects of amino acids after weaning." In 6th EAAP International Symposium on Energy and Protein Metabolism and Nutrition. The Netherlands: Wageningen Academic Publishers, 2019. http://dx.doi.org/10.3920/978-90-8686-891-9_98.

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ISMAILOV, Ismail Sagidovich, Nina Vladimirovna TREGUBOVA, Rashid Hasanbievich KOCHKAROV, Anna Victorovna MORGUNOVA, and Natalija Alecseevna DRIZHD. "THE RELATIONSHEEP OF AMINO ACID METABOLISM WITH PRODUCTIVITY OF GROWING YOUNG SHEEP." In RURAL DEVELOPMENT. Aleksandras Stulginskis University, 2018. http://dx.doi.org/10.15544/rd.2017.125.

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A number of studies on digestion of the ruminants have shown the process of synthesis of all the essential amino acids in the rumen (Abu Fadel, 2004; Trukhachev, V., Zlidnev, N. 2008). However, according to some researches, microbial protein is deficient in methionine and lysine. This assumption also proves the need for further study of the influence of balanced amino acids to these diets on the growth, development and productive performance of the ruminants. Scientific approbation of the issues related to establishing the requirements of young sheep in lysine and methionine with cystine and development of recommendations for their proper balance in animal rations have been conducted along with a series of physiological, scientific and practical experiments. For this purpose, we used the following research methods: preparative, analytical, measuring and calculating. Zootechnical evaluation of young sheep’s diet with different levels of lysine and methionine with cysteine has been conducted, and influence of these components on growth, development, metabolism, the use of amino acids and wool productivity have been studied. The importance of studying the content of amino acids in plasma is evident because they are the indicators of protein metabolism in the animals’ organism and represent themselves the exchange fund when used in the biochemical transformations in the process of updating the protein of the body tissues and the synthesis of animal products. Increase in young sheep’s diet of lysine and methionine can improve their productivity, reduce the cost of feed energy and improve biochemical indexes of meat, taking into account the optimization of its amino acid composition. Efficiency increase of young sheep during the process of the individual development is linked to conditions of feeding and in particular the usefulness of a protein food, which is primarily determined by sufficient intake of essential amino acids - lysine and methionine with cystine. Thus, the inclusion in the diet of growing young sheep some amount of synthetic amino acids – 6–8 grams of lysine and 3–4 g of methionine per 1 fodder unit, enhances oxidation-reduction processes, increase productivity, i.e. wool yield and other indicators. The content of free amino acids in plasma directly depends on their availability in feed. The use of synthetic amino acids (lysine, methionine and cystine) contributed to the increase of their content in a free state in the blood serum, indicating their increased demand in sheep. With the use of different doses of synthetic amino acids (lysine, methionine, cystine) in the diets of young sheep it has been found that the organization of optimum conditions of feeding, care and management of sheep in winter season help to avoid abrupt changes in the structure of the skin, to ensure the normal development of the histological structure and morphogenetic processes.)

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Valadares Filho, S. C., P. M. Amaral, D. Zanetti, L. D. S. Mariz, S. A. Santos, S. A. Lopes, L. F. Prados, et al. "Net essential amino acids requirements for Nellore and crossbred Angus × Nellore cattle." In 6th EAAP International Symposium on Energy and Protein Metabolism and Nutrition. The Netherlands: Wageningen Academic Publishers, 2019. http://dx.doi.org/10.3920/978-90-8686-891-9_151.

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Fernández-Fígares, I., L. Lara, and M. Lachica. "Betaine increases portal appearance of amino acids and peptides in Iberian pigs." In 6th EAAP International Symposium on Energy and Protein Metabolism and Nutrition. The Netherlands: Wageningen Academic Publishers, 2019. http://dx.doi.org/10.3920/978-90-8686-891-9_77.

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Lærke, H. N., A. K. Ingerslev, P. Zhou, J. V. Nørgaard, and S. K. Jensen. "Milk protein – more than just amino acids? A study on growth and growth biomarkers in piglets." In 6th EAAP International Symposium on Energy and Protein Metabolism and Nutrition. The Netherlands: Wageningen Academic Publishers, 2019. http://dx.doi.org/10.3920/978-90-8686-891-9_110.

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Rabbani, Naila, and Paul John Thornalley. "Blood Biomarkers associated with Autism Spectrum Disorder may Provide Early Diagnosis." In Qatar University Annual Research Forum & Exhibition. Qatar University Press, 2020. http://dx.doi.org/10.29117/quarfe.2020.0151.

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Autism spectrum disorders (ASD) are a collection of neuropsychiatric disorders. ASD affects 1 in 87 in Qatar and is expected to increase. The high variability and heterogeneity of the symptoms makes diagnosis of ASD difficult and uncertain, particularly at the early stages of development. If detected early, clinical support can be given to promote optimal development and well-being of children with ASD and even achieve complete remission. The current method of diagnosing ASD is by observations and interviews made by experts in child development to assess child behavior, communication and cognition: the ADOS test. There is often delay in referral for expert diagnosis; delay is typically >18 months in Qatar and >4 years in EU and USA. The diagnostic accuracy is 60 – 70%. Mutations in proteins transporters of amino acids have been linked to some cases of ASD. We hypothesized that levels of amino acids in blood, including amino acids damaged by modification by sugars (glycation), oxidation and nitration may provide novel biomarker for diagnosis of ASD at very young age.

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Hosios, Aaron M., Vivian C. Hecht, Marc Johnson, Jeffrey C. Rathmell, Scott R. Manalis, and Matthew G. Vander Heiden. "Abstract A36: Amino acids rather than glucose accounts for the majority of cell mass in rapidly proliferating mammalian cells." In Abstracts: AACR Special Conference: Metabolism and Cancer; June 7-10, 2015; Bellevue, WA. American Association for Cancer Research, 2016. http://dx.doi.org/10.1158/1557-3125.metca15-a36.

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Reports on the topic "Amino acids Metabolism Disorders"

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Blumwald, Eduardo, and Avi Sadka. Sugar and Acid Homeostasis in Citrus Fruit. United States Department of Agriculture, January 2012. http://dx.doi.org/10.32747/2012.7697109.bard.

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Citrus fruit quality standards have been determined empirically, depending on species and on the particular growing regions. In general, the TSS (total soluble solids) to total acidity (TA) ratio determines whether citrus fruit can be marketed. Soluble sugars account for most of the TSS during harvest while TA is determined almost solely by the citric acid content, which reaches levels of 1-5% by weight in many cultivated varieties. Acid and sugar homeostasis in the fruit is critical for the management of existing cultivars, the development of new cultivars, the improvement of pre- and post-harvest strategies and the control of fruit quality and disorders. The current proposal (a continuation of a previous proposal) aimed at: (1) completing the citrus fruit proteome and metabolome, and establish a citrus fruit functional database, (2) further characterization of the control of fruit acidity by studying the regulation of key steps affecting citrate metabolism, and determine the fate of citrate during acid decline stage, and (3) Studying acid and sugar homeostasis in citrus fruits by characterizing transport mechanisms across membranes. These aims were completed as the following: (1) Our initial efforts were aimed at the characterization and identification of citric acid transporters in citrus juice cells. The identification of citrate transporters at the vacuole of the citrus juice cell indicated that the steady-state citrate cytosolic concentration and the action of the cytosolic aconitase were key elements in establishing the pH homeostat in the cell that regulates the metabolic shift towards carbon usage in the fruit during the later stages of fruit development. We focused on the action of aconitase, the enzyme mediating the metabolic use of citric acid in the cells, and identified processes that control carbon fluxes in developing citrus fruits that control the fruit acid load; (2) The regulation of aconitase, catalyzing a key step in citrate metabolism, was further characterized by using two inhibitors, citramalte and oxalomalte. These compounds significantly increased citrate content and reduced the enzyme’s activity. Metabolite profiling and changes of amino-acid metabolizing enzymes in oxalomalate- treated cells suggested that the increase in citrate, caused by aconitase inhibition, induces amino acid synthesis and the GABA shunt, in accordance with the suggested fate of citrate during the acid decline stage in citrus fruit. (3) We have placed a considerable amount of time on the development of a citrus fruit proteome that will serve to identify all of the proteins in the juice cells and will also serve as an aid to the genomics efforts of the citrus research community (validating the annotation of the fruit genes and the different ESTs). Initially, we identified more than 2,500 specific fruit proteins and were able to assign a function to more than 2,100 proteins (Katz et al., 2007). We have now developed a novel Differential Quantitative LC-MS/MS Proteomics Methodology for the identification and quantitation of key biochemical pathways in fruits (Katz et al., 2010) and applied this methodology to identify determinants of key traits for fruit quality (Katz et al., 2011). We built “biosynthesis maps” that will aid in defining key pathways associated with the development of key fruit quality traits. In addition, we constructed iCitrus (http://wiki.bioinformatics.ucdavis.edu/index.php/ICitrus), a “functional database” that is essentially a web interface to a look-up table that allows users to use functional annotations in the web to identify poorly annotated citrus proteins. This resource will serve as a tool for growers and field extension specialists.

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Anderson, Olin D., Gad Galili, and Ann E. Blechl. Enhancement of Essential Amino Acids in Cereal Seeds: Four Approaches to Increased Lysine Content. United States Department of Agriculture, October 1998. http://dx.doi.org/10.32747/1998.7585192.bard.

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Cereal seeds are the basis of the human diet, and their amino acid composition is thus of major nutritional and economic importance. Currently, deficiencies in essential amino acids are addressed, when possible, by additionalprotein sources or by supplementing animal feed with non-cereal protein or synthetic amino acids. A number of strategies have been suggested to make cereal flours more complete and balanced sources of amino acids, although systematic examination of such strategies is rare. This project proposed to begin such a systematic examination using four complementary and parallel approaches to increasing wheat seed lysine: 1) Modifying endogenous wheat seed proteins for increased lysine composition. 2) Overexpression of naturally occurring high-lysine proteins in the wheat endosperm. 3) Ectopic expression of proteins in the wheat endosperm. 4) Alteration of free lysine levels in the wheat endosperm. The results of these studies are expected to be wheat lines with increased lysine content and will establish a clearer understanding of the approaches most likely to enhance cereal seed protein quality. Progress is reported for all four objectives, with a significant foundation for further work on two of the objectives (modification of wheat storage proteins and lysine metabolism). Plans for continuing work on all four objectives are briefly outlined.

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Varga, Gabriella A., Amichai Arieli, Lawrence D. Muller, Haim Tagari, Israel Bruckental, and Yair Aharoni. Effect of Rumen Available Protein, Amimo Acids and Carbohydrates on Microbial Protein Synthesis, Amino Acid Flow and Performance of High Yielding Cows. United States Department of Agriculture, August 1993. http://dx.doi.org/10.32747/1993.7568103.bard.

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The effect of rumen available protein amino acids and carbohydrates on microbial protein synthesis, amino acid flow and performance of high yielding dairy cows was studied. A significant relationship between the effective degradabilities of OM in feedstuffs and the in vivo ruminal OM degradation of diets of dairy cows was found. The in situ method enabled the prediction of ruminal nutrients degradability response to processing of energy and nitragenous supplements. The AA profile of the rumen undegradable protein was modified by the processing method. In a continuous culture study total N and postruminal AA flows, and bacterial efficiency, is maximal at rumen degradable levels of 65% of the CP. Responses to rumen degradable non carbohydrate (NSC) were linear up to at least 27% of DM. Higher CP flow in the abomasum was found for cows fed high ruminally degradable OM and low ruminally degradable CP diet. It appeared that in dairy cows diets, the ratio of rumen degradable OM to rumenally degradable CP should be at least 5:1 in order to maximize postruminal CP flow. The efficiency of microbial CP synthesis was higher for diets supplemented with 33% of rumen undegradable protein, with greater amounts of bacterial AA reaching the abomasum. Increase in ruminal carbohydrate availability by using high moisture corn increased proportions of propionate, postruminal nutrients flow, postruminal starch digestibility, ruminal availability of NSC, uptake of energy substrates by the mammory gland. These modifications resulted with improvement in the utilization of nonessential AA for milk protein synthesis, in higher milk protein yield. Higher postruminal NSC digestibility and higher efficiency of milk protein production were recorded in cows fed extruded corn. Increasing feeding frequency increased flow of N from the rumen to the blood, reduced diurnal variation in ruminal and ammonia, and of plasma urea and improved postruminal NSC and CIP digestibility and total tract digestibilities. Milk and constituent yield increased with more frequent feeding. In a study performed in a commercial dairy herd, changes in energy and nitrogenous substrates level suggested that increasing feeding frequency may improve dietary nitrogen utilization and may shift metabolism toward more glucogenesis. It was concluded that efficiency of milk protein yield in high producing cows might be improved by an optimization of ruminal and post-ruminal supplies of energy and nitrogenous substrates. Such an optimization can be achieved by processing of energy and nitrogenous feedstuffs, and by increasing feeding frequency. In situ data may provide means for elucidation of the optimal processing conditions.

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Galili, Gad, Harry J. Klee, and Asaph Aharoni. Elucidating the impact of enhanced conversion of primary to secondary metabolism on phenylpropanoids secondary metabolites associated with flavor, aroma and health in tomato fruits. United States Department of Agriculture, January 2012. http://dx.doi.org/10.32747/2012.7597920.bard.

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• Targeted manipulating Phenylalanine (Phe) synthesis is one of the most powerful strategies to boost the biologically and economically important secondary metabolites, including phenylpropaniods, aromatic volatiles and specialized secondary metabolites. • Over-expression of the petunia MYB transcript factor, ODORANT1 (ODO1), results in significant alterations of the levels of specific phenylpropanoid compounds in plants. • Our previous studies indicated that ectopic expression of the feedback-insensitive AroG could break the bottleneck between primary and secondary metabolisms in tomato, thereby aiding in producing new tomato composition and identifying the unknown roles of multiple key regulators in specialized metabolism. Therefore, combining the AroG and ODO1 is of particular interest for elucidating the combined regulatory role of both of these genes in the Phe metabolic pathway, as well as generating tomato fruits that contain higher levels of secondary metabolites. • Here, we performed the LC-MS and GC-MS analyses on fruits of four tomato genotypes, namely, wild type tomato fruits as well as tomato fruits expressing the AroG, ODO1 and the combination of AroG plus ODO1 (AO) genotypes. Our results elaborated that the levels of many of the Phe-derived metabolites were predominately altered in fruits of the AO genotype, compared to tomato fruits expressing either AroG or ODO1 individually. The levels of most of these metabolites were significantly stimulated, such as Tyrosine (Tyr), coumaric acid and ferulic acid derived metabolites, but the levels of some important secondary metabolites were reduced in the AO transgenic genotypes as compared to either AroG or ODO1 lines. Nevertheless, our results also revealed that the levels of aromatic volatiles were obviously down regulated in the AO, compared to that in AroG transgenic fruits, but were boosted while compared to the wild type and ODO1 transgenic fruits. • Our results suggest that ODO1 expression may also have a negative effect on the production of some of the aromatic volatiles in tomato fruits, indicating that ODO1 acts as an important regulator of the shikimate pathway, which leads to the production of the aromatic amino acids and secondary metabolites derived from them. Key words: AroG, ODO1, tomato, metabolism, shikimate pathway

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Jander, Georg, Gad Galili, and Yair Shachar-Hill. Genetic, Genomic and Biochemical Analysis of Arabidopsis Threonine Aldolase and Associated Molecular and Metabolic Networks. United States Department of Agriculture, January 2010. http://dx.doi.org/10.32747/2010.7696546.bard.

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Since the amino acids threonine and isoleucine can be limiting in mammalian diet and there is interest in increasing their abundance in certain crop plants. To meet this need, a BARD proposal was written with two main research objectives: (i) investigate new avenues for manipulating threonine and isoleucine content in plants and (ii) study the role of threonine aldolase in plant metabolism. Research conducted to meet these goals included analysis of the sub-cellular localization of threonine aldolase in the plant, analysis of metabolic flux in developing embryos, over- and under-expression of Arabidopsis threonine aldolases, and transcriptional and metabolic analysis of perturbations resulting from altered threonine aldolase expression. Additionally, the broader metabolic effects of increasing lysine biosynthesis were investigated. An interesting observation that came up in the course of the project is that threonine aldolase activity affects methionine gamma-lyase in Arabidopsis. Further research showed that threonine deaminase and methionine gamma-lyase both contribute to isoleucine biosynthesis in plants. Therefore, isoleucine content can be altered by manipulating the expression of either or both of these enzymes. Additionally, both enzymes contribute to the up to 100-fold increase in isoleucine that is observed in drought-stressed Arabidopsis. Toward the end of the project it was discovered that through different projects, both groups had been able to independently up-regulate phenylalanine accumulation by different mechanisms. The Galili lab transformed Arabidopsis with a feedbackinsensitive bacterial enzyme and the Jander lab found a feedback insensitive mutation in Arabidopsis arogenate dehydratase. Exchange of the respective plant lines has allowed a comparative analysis of the different methods for increasing phenylalanine content and the creation of double mutants. The research that was conducted as part of this BARD project has led to new insights into plant amino acid metabolism. Additionally, new approaches that were found to increase the accumulation of threonine, isoleucine, and phenylalanine in plants have potential practical applications. Increased threonine and isoleucine levels can increase the nutritional value of crop plants. Elevated isoleucine accumulation may increase the osmotic stress tolerance of plants. Up-regulation of phenylalanine biosynthesis can be used to increase the production of downstream higher-value plant metabolites of biofuel feed stocks.

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Whitham, Steven A., Amit Gal-On, and Tzahi Arazi. Functional analysis of virus and host components that mediate potyvirus-induced diseases. United States Department of Agriculture, March 2008. http://dx.doi.org/10.32747/2008.7591732.bard.

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The mechanisms underlying the development of symptoms in response to virus infection remain to be discovered in plants. Insight into symptoms induced by potyviruses comes from evidence implicating the potyviral HC-Pro protein in symptom development. In particular, recent studies link the development of symptoms in infected plants to HC-Pro's ability to interfere with small RNA metabolism and function in plant hosts. Moreover, mutation of the highly conserved FRNK amino acid motif to FINK in the HC-Pro of Zucchini yellow mosaic virus (ZYMV) converts a severe strain into an asymptomatic strain, but does not affect virus accumulation in cucurbit hosts. The ability of this FINK mutation to uncouple symptoms from virus accumulation creates a unique opportunity to study symptom etiology, which is usually confounded by simultaneous attenuation of both symptoms and virus accumulation. Our goal was to determine how mutations in the conserved FRNK motif affect host responses to potyvirus infection in cucurbits and Arabidopsis thaliana. Our first objective was to define those amino acids in the FRNK motif that are required for symptoms by mutating the FRNK motif in ZYMV and Turnip mosaic virus (TuMV). Symptom expression and accumulation of resulting mutant viruses in cucurbits and Arabidopsis was determined. Our second objective was to identify plant genes associated with virus disease symptoms by profiling gene expression in cucurbits and Arabidopsis in response to mutant and wild type ZYMV and TuMV, respectively. Genes from the two host species that are differentially expressed led us to focus on a subset of genes that are expected to be involved in symptom expression. Our third objective was to determine the functions of small RNA species in response to mutant and wild type HC-Pro protein expression by monitoring the accumulation of small RNAs and their targets in Arabidopsis and cucurbit plants infected with wild type and mutant TuMV and ZYMV, respectively. We have found that the maintenance of the charge of the amino acids in the FRNK motif of HC-Pro is required for symptom expression. Reduced charge (FRNA, FRNL) lessen virus symptoms, and maintain the suppression of RNA silencing. The FRNK motif is involved in binding of small RNA species including microRNAs (miRNA) and short interfering RNAs (siRNA). This binding activity mediated by the FRNK motif has a role in protecting the viral genome from degradation by the host RNA silencing system. However, it also provides a mechanism by which the FRNK motif participates in inducing the symptoms of viral infection. Small RNA species, such as miRNA and siRNA, can regulate the functions of plant genes that affect plant growth and development. Thus, this binding activity suggests a mechanism by which ZYMVHC-Pro can interfere with plant development resulting in disease symptoms. Because the host genes regulated by small RNAs are known, we have identified candidate host genes that are expected to play a role in symptoms when their regulation is disrupted during viral infections. As a result of this work, we have a better understanding of the FRNK amino acid motif of HC-Pro and its contribution to the functions of HC-Pro, and we have identified plant genes that potentially contribute to symptoms of virus infected plants when their expression becomes misregulated during potyviral infections. The results set the stage to establish the roles of specific host genes in viral pathogenicity. The potential benefits include the development of novel strategies for controlling diseases caused by viruses, methods to ensure stable expression of transgenes in genetically improved crops, and improved potyvirus vectors for expression of proteins or peptides in plants.

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Dudareva, Natalia, Alexander Vainstein, Eran Pichersky, and David Weiss. Integrating biochemical and genomic approaches to elucidate C6-C2 volatile production: improvement of floral scent and fruit aroma. United States Department of Agriculture, September 2007. http://dx.doi.org/10.32747/2007.7696514.bard.

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The specific objectives of approved proposal include to: 1. Elucidate the C6-C2 biochemical pathways leading to the biosynthesis of phenylacetaldehyde, phenylethyl alcohol and phenylethyl acetate in floral tissues of ornamentally important plants, pefunia and roses. 2. Isolate and characterrze genes responsible for the production of these C6-C2 compounds and those involved in the regulation of the pathway using genomic and transcriptomic tools. 3. Determine whether altering the expression of key genes of this pathway can result in changing the aroma characteristics of flowers. Aldehydes are intermediates in a variety of biochemical pathways including those involved in the metabolism of carbohydrates, vitamins, steroids, amino acids, benzylisoquinoline alkaloids, hormones, and lipids. In plants they are also synthesized in response to environmental stresses such as salinity, cold, and heat shock or as flavors and aromas in fruits and flowers. Phenylacetaldehyde along with 2-phenylethanol and its acetate ester, are important scent compounds in numerous flowers, including petunias and roses. However, little is known about the biosynthesis of these volatile compounds in plants. We have shown that the formation PHA and 2-phenylethanol from Phe does not occur via trans-cinnamic acid and instead competes with the key enzyme of phenypropanoid metabolism Pheammonia-lyase (PAL) for Phe utilization. Using functional genomic approach and comparative gene expression profiling, we have isolated and characterized a novel enzyme from petunia and rose flowers that catalyzes the formation of the Ca-Czcompound phenylacetaldehyde (PHA) from L-phenylalanine (Phe) by the removal of both the carboxyl and amino groups. This enzyme, designated as phenylacetaldehyde synthases (PAAS), is a bifunctional enzyme that catalyzes the unprecedented efficient coupling of phenylalanine decarboxylation to oxidation, generating phenylacetaldehyde, CO2, ammonia, and hydrogen peroxide in stoichiometric amounts. Down-regulation of PAAS expression via RNA interference-based (RNAi) technology in petunia resulted in no PHA emission when compared with controls. These plants also produced no 2-phenylethanol, supporting our conclusion that PHA is a precursor of 2-phenylethanol. To understand the regulation of scent formation in plants we have also generated transgenic petunia and tobacco plants expressing the rose alcohol acetyltransferase (RhAAT) gene under the control of a CaMV-35S promoter. Although the preferred substrate of RhAAT in vitro is geraniol, in transgenic petunia flowers, it used phenylethyl alcohol and benzyl alcohol to produce the corresponding acetate esters, not generated by control flowers. These results strongly point to the dependence of volatile production on substrate availability. Analysis of the diurnal regulation of scent production in rose flowers revealed that although the daily emission of most scent compounds is synchronized, various independently evolved mechanisms control the production, accumulation and release of different volatiles. This research resulted in a fundamental discovery of biochemical pathway, enzymes and genes involved in biosynthesis of C6-C2s compounds, and provided the knowledge for future engineering plants for improved scent quality.

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