Literatura académica sobre el tema "Long-chain-acyl-CoA dehydrogenase"
Crea una cita precisa en los estilos APA, MLA, Chicago, Harvard y otros
Consulte las listas temáticas de artículos, libros, tesis, actas de conferencias y otras fuentes académicas sobre el tema "Long-chain-acyl-CoA dehydrogenase".
Junto a cada fuente en la lista de referencias hay un botón "Agregar a la bibliografía". Pulsa este botón, y generaremos automáticamente la referencia bibliográfica para la obra elegida en el estilo de cita que necesites: APA, MLA, Harvard, Vancouver, Chicago, etc.
También puede descargar el texto completo de la publicación académica en formato pdf y leer en línea su resumen siempre que esté disponible en los metadatos.
Artículos de revistas sobre el tema "Long-chain-acyl-CoA dehydrogenase"
Masterson, C., A. Blackburn y C. Wood. "Acyl-CoA dehydrogenase activity in pea cotyledon tissue during germination and initial growth". Biochemical Society Transactions 28, n.º 6 (1 de diciembre de 2000): 760–62. http://dx.doi.org/10.1042/bst0280760.
Texto completoTreem, William R., Jeffrey S. Hyams, Charles A. Stanley, Daniel E. Hale y Harris B. Leopold. "Hypoglycemia, Hypotonia, and Cardiomyopathy: The Evolving Clinical Picture of Long-Chain Acyl-CoA Dehydrogenase Deficiency". Pediatrics 87, n.º 3 (1 de marzo de 1991): 328–33. http://dx.doi.org/10.1542/peds.87.3.328.
Texto completoCox, Keith B., Jian Liu, Liqun Tian, Stephen Barnes, Qinglin Yang y Philip A. Wood. "Cardiac hypertrophy in mice with long-chain acyl-CoA dehydrogenase or very long-chain acyl-CoA dehydrogenase deficiency". Laboratory Investigation 89, n.º 12 (7 de septiembre de 2009): 1348–54. http://dx.doi.org/10.1038/labinvest.2009.86.
Texto completoYu, Wenfeng, Xiquan Liang, Regina E. Ensenauer, Jerry Vockley, Lawrence Sweetman y Horst Schulz. "Leaky β-Oxidation of atrans-Fatty Acid". Journal of Biological Chemistry 279, n.º 50 (4 de octubre de 2004): 52160–67. http://dx.doi.org/10.1074/jbc.m409640200.
Texto completoWijayabandara, Maheshi, Champika Gamakaranage y Dineshani Hettiarachchi. "Very-Long-Chain Acyl-Co-Enzyme A Dehydrogenase Deficiency Presenting as Rhabdomyolysis: First Case Report from Sri Lanka". Case Reports in Genetics 2020 (13 de octubre de 2020): 1–5. http://dx.doi.org/10.1155/2020/8894518.
Texto completoLiang, X., W. Le, D. Zhang y H. Schulz. "Impact of the intramitochondrial enzyme organization on fatty acid oxidation". Biochemical Society Transactions 29, n.º 2 (1 de mayo de 2001): 279–82. http://dx.doi.org/10.1042/bst0290279.
Texto completoYamaguchi, Seiji, Yasuhiro Indo, Paul M. Coates, Takashi Hashimoto y Kay Tanaka. "Identification of Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency in Three Patients Previously Diagnosed with Long-Chain Acyl-CoA Dehydrogenase Deficiency". Pediatric Research 34, n.º 1 (julio de 1993): 111–13. http://dx.doi.org/10.1203/00006450-199307000-00025.
Texto completoCosta, Catarina G., Lambertus Dorland, Ulbe Holwerda, Isabel Tavares de Almeida, Bwee-Tien Poll-The, Cornelis Jakobs y Marinus Duran. "Simultaneous analysis of plasma free fatty acids and their 3-hydroxy analogs in fatty acid β-oxidation disorders". Clinical Chemistry 44, n.º 3 (1 de marzo de 1998): 463–71. http://dx.doi.org/10.1093/clinchem/44.3.463.
Texto completoNandy, Andreas, Volker Kieweg, Franz-Georg Kräutle, Petra Vock, Burkhard Küchler, Peter Bross, Jung-Ja P. Kim, Ihab Rasched y Sandro Ghisla. "Medium-Long-Chain Chimeric Human Acyl-CoA Dehydrogenase: Medium-Chain Enzyme with the Active Center Base Arrangement of Long-Chain Acyl-CoA Dehydrogenase†". Biochemistry 35, n.º 38 (enero de 1996): 12402–11. http://dx.doi.org/10.1021/bi960785e.
Texto completoParsons, H. G. y V. C. Dias. "Intramitochondrial fatty acid metabolism: riboflavin deficiency and energy production". Biochemistry and Cell Biology 69, n.º 7 (1 de julio de 1991): 490–97. http://dx.doi.org/10.1139/o91-073.
Texto completoTesis sobre el tema "Long-chain-acyl-CoA dehydrogenase"
Keeler, Allison M. "Gene Therapy for Very Long Chain Acyl-coA Dehydrogenase Deficiency Using Adeno-Associated Virus Vectors: A Dissertation". eScholarship@UMMS, 2012. https://escholarship.umassmed.edu/gsbs_diss/632.
Texto completoKrogmann, Antonia Verfasser], Ute [Gutachter] [Spiekerkötter y Anselmino Verena [Gutachter] Keitel-. "Der renale Phänotyp beim Very-Long-Chain Acyl-CoA Dehydrogenase- (VLCAD-) Mangel: Studien am VLCAD-/--Mausmodell / Antonia Krogmann ; Gutachter: Ute Spiekerkötter, Verena Keitel- Anselmino". Düsseldorf : Universitäts- und Landesbibliothek der Heinrich-Heine-Universität Düsseldorf, 2020. http://d-nb.info/1217062300/34.
Texto completoWang, Xiao. "Investigation of Anaplerosis from Propionyl-CoA Precursors and Fatty Acid Oxidation in the Brain of VLCAD and Control Mice". Case Western Reserve University School of Graduate Studies / OhioLINK, 2009. http://rave.ohiolink.edu/etdc/view?acc_num=case1232676063.
Texto completoTawbeh, Ali. "Dysfonctionnement peroxysomal dans les cellules microgliales BV-2 : vers une meilleure compréhension du processus neurodégénératif dans l'adrénoleucodystrophie liée à l'X". Electronic Thesis or Diss., Bourgogne Franche-Comté, 2023. http://www.theses.fr/2023UBFCI010.
Texto completoMicroglia act as the immune sentinel of the central nervous system and play a crucial role in maintaining its homeostasis. Peroxisomes, essential organelles in cellular metabolism, mainly orchestrate the breakdown of very long-chain fatty acids (VLCFAs). The accumulation of VLCFAs is a marker of peroxisomal diseases, the most common of which is X-linked adrenoleukodystrophy (X-ALD), a rare and complex neurodegenerative disease in which microglia are thought to play an important role. The inactivation of peroxisomal genes in BV-2 microglial cells had shed light on a yet unexplored relationship between peroxisomal activity and microglial plasticity. Specifically, knocked-out genes included those encoding the very long-chain fatty acid transporters ABCD1 and ABCD2, as well as the peroxisomal β-oxidation-limiting enzyme ACOX1. Transcriptomic and functional analysis of the mutant cells revealed a disease-associated microglial signature close to that found in the most common neurodegenerative diseases. The peroxisomal defect impacts lipid metabolism in the broad sense, lysosomal and autophagic functions, as well as the response to lipopolysaccharide stimulation, including the inflammatory response and redox homeostasis. The functions of phagocytosis and antigen presentation to T lymphocytes are also altered. In addition, secretions from these mutant microglial cells induce the death of neuronal and oligodendrocyte cell lines and modify the morphology and function of neurons. Although these results need to be confirmed in primary microglia, they support the hypothesis of a major and early role for microglia in the neurodegenerative cascade observed in X-ALD and validate microglia and the molecules they secrete as a therapeutic target in peroxisomal leukodystrophies
Celorico, Ana Patrícia Monteiro. "Very long-chain Acyl-CoA dehydrogenase deficiency from gene to treatment". Master's thesis, 2015. http://hdl.handle.net/10451/26955.
Texto completoA ß-oxidação mitocondrial dos ácidos gordos (MFAO) é uma importante fonte de energia no músculo, especialmente quando as necessidades energéticas fisiológicas estão aumentadas. A deficiência da desidrogenase de ésteres acil-CoA de cadeia muito longa (VLCAD) é caracterizada por uma diminuição da MFAO. A VLCAD é uma das quatro desidrogenases de ésteres acil-CoA que catalisam o primeiro passo da MFAO. A VLCAD é um homodímero de 67 kDa associado à membrana interna da mitocôndria e codificado pelo gene ACADVL. O gene ACADVL, com cerca de 5,4 Kb, está localizado no cromossoma 17p13.1 e contém 2177 bases codificantes. A deficiência em VLCAD (VLCADD) é uma doença clinicamente heterogénea que apresenta três grupos clínicos principais: uma forma cardíaca grave com início na infância, uma forma hipoglicemiante mais ligeira com início na infância e uma forma miopática com início na adolescência/idade adulta. O diagnóstico é muitas vezes complicado devido às características clínicas serem comuns a todas as miopatias metabólicas. Este baseia-se no fenótipo clínico, testes bioquímicos, testes genéticos, e /ou avaliação post-mortem. O número de doentes diagnosticados com VLCADD tem aumentado, no entanto, os estudos ainda são limitados. Os tratamentos descritos consistem em: evitar jejum prolongado; perfusão com glucose no caso de crises metabólicas; terapêutica com carnitina e dieta restrita em ácidos de cadeia longa e rica em hidratos de carbono. Trabalhos recentes apontam ainda para o potencial terapêutico do resveratrol. A terapêutica génica foi já testada in vivo em modelos animais.
Oliveira, Ana Sofia Gonçalves Novo e. Rodrigues de. "Pregnancy in very long-chain acyl-CoA dehydrogenase deficiency: maternal-fetal risk (case report)". Master's thesis, 2020. https://hdl.handle.net/10216/127929.
Texto completoOliveira, Ana Sofia Gonçalves Novo e. Rodrigues de. "Pregnancy in very long-chain acyl-CoA dehydrogenase deficiency: maternal-fetal risk (case report)". Dissertação, 2020. https://hdl.handle.net/10216/127929.
Texto completoSchymik, Ina [Verfasser]. "Der Very-long-chain-Acyl-CoA-Dehydrogenase-(VLCAD)-Mangel in Deutschland / vorgelegt von Ina Schymik". 2008. http://d-nb.info/990026132/34.
Texto completoCapítulos de libros sobre el tema "Long-chain-acyl-CoA dehydrogenase"
Schomburg, Dietmar, Margit Salzmann y Dörte Stephan. "Long-chain-acyl-CoA dehydrogenase". En Enzyme Handbook, 687–89. Berlin, Heidelberg: Springer Berlin Heidelberg, 1993. http://dx.doi.org/10.1007/978-3-642-58051-2_145.
Texto completoLeung, Alexander K. C., William Lane M. Robson, Carsten Büning, Johann Ockenga, Janine Büttner, Hartmut Schmidt, Antonio V. Delgado-Escueta et al. "Long-Chain Acyl-CoA Dehydrogenase Deficiency". En Encyclopedia of Molecular Mechanisms of Disease, 1210. Berlin, Heidelberg: Springer Berlin Heidelberg, 2009. http://dx.doi.org/10.1007/978-3-540-29676-8_7545.
Texto completoBalemans, Wendy, Wim Van Hul, Marian Valko, Jan Moncol, Lee A. Denson, Maria Mela, Ulrich Thalheimer et al. "Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency". En Encyclopedia of Molecular Mechanisms of Disease, 2202–4. Berlin, Heidelberg: Springer Berlin Heidelberg, 2009. http://dx.doi.org/10.1007/978-3-540-29676-8_3151.
Texto completoObaid, Abdulrahman, Marwan Nashabat, Majid Alfadhel, Ali Alasmari, Fuad Al Mutairi, Abdulrahman Alswaid, Eissa Faqeih et al. "Clinical, Biochemical, and Molecular Features in 37 Saudi Patients with Very Long Chain Acyl CoA Dehydrogenase Deficiency". En JIMD Reports, 47–53. Berlin, Heidelberg: Springer Berlin Heidelberg, 2017. http://dx.doi.org/10.1007/8904_2017_58.
Texto completoSpiekerkoetter, U., M. Mueller, M. Sturm, M. Hofmann y D. T. Schneider. "Lethal Undiagnosed Very Long-Chain Acyl-CoA Dehydrogenase Deficiency with Mild C14-Acylcarnitine Abnormalities on Newborn Screening". En JIMD Reports, 113–15. Berlin, Heidelberg: Springer Berlin Heidelberg, 2012. http://dx.doi.org/10.1007/8904_2012_129.
Texto completoDe Biase, Irene, Krista S. Viau, Aiping Liu, Tatiana Yuzyuk, Lorenzo D. Botto, Marzia Pasquali y Nicola Longo. "Diagnosis, Treatment, and Clinical Outcome of Patients with Mitochondrial Trifunctional Protein/Long-Chain 3-Hydroxy Acyl-CoA Dehydrogenase Deficiency". En JIMD Reports, 63–71. Berlin, Heidelberg: Springer Berlin Heidelberg, 2016. http://dx.doi.org/10.1007/8904_2016_558.
Texto completoBouvier, Damien, Christine Vianey-Saban, Séverine Ruet y Cécile Acquaviva. "Development of a Tandem Mass Spectrometry Method for Rapid Measurement of Medium- and Very-Long-Chain Acyl-CoA Dehydrogenase Activity in Fibroblasts". En JIMD Reports, 71–78. Berlin, Heidelberg: Springer Berlin Heidelberg, 2016. http://dx.doi.org/10.1007/8904_2016_22.
Texto completoMerinero, B., P. Alcaide, E. Martín-Hernández, A. Morais, M. T. García-Silva, P. Quijada-Fraile, C. Pedrón-Giner et al. "Four Years’ Experience in the Diagnosis of Very Long-Chain Acyl-CoA Dehydrogenase Deficiency in Infants Detected in Three Spanish Newborn Screening Centers". En JIMD Reports, 63–74. Berlin, Heidelberg: Springer Berlin Heidelberg, 2017. http://dx.doi.org/10.1007/8904_2017_40.
Texto completoKarall, D., G. Mair, U. Albrecht, K. Niedermayr, T. Karall, W. Schobersberger y S. Scholl-Bürgi. "Sports in LCHAD Deficiency: Maximal Incremental and Endurance Exercise Tests in a 13-Year-Old Patient with Long-Chain 3-Hydroxy Acyl-CoA Dehydrogenase Deficiency (LCHADD) and Heptanoate Treatment". En JIMD Reports, 7–12. Berlin, Heidelberg: Springer Berlin Heidelberg, 2014. http://dx.doi.org/10.1007/8904_2014_313.
Texto completo"Very long chain acyl CoA dehydrogenase deficiency". En Atlas of Inherited Metabolic Diseases 3E, 289–94. CRC Press, 2011. http://dx.doi.org/10.1201/b15310-44.
Texto completo