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1

Quant, Patti A., and Simon Eaton, eds. Current Views of Fatty Acid Oxidation and Ketogenesis. Boston, MA: Springer US, 2002. http://dx.doi.org/10.1007/b113063.

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2

International Symposium on Clinical, Biochemical and Molecular Aspects of Fatty Acid Oxidation (1988 Philadelphia, Pa.). Fatty acid oxidation: Clinical biochemical, and molecular aspects : proceedings of the International Symposium on Clinical, Biochemical and Molecular Aspects of Fatty Acid Oxidation, held November 6-9, 1988 in Philadelphia. Edited by Tanaka Kay and Coates Paul M. New York: Liss, 1989.

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3

M, Coates Paul, and Tanaka Kay, eds. New developments in fatty acid oxidation: Proceedings of the Second International Symposium on Clinical, Biochemical, and Molecular Aspects of Fatty Acid Oxidation, held in Philadelphia, Pennsylvania, November 1991. New York, N.Y: Wiley-Liss, 1992.

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4

Kay, Tanaka, and Coates Paul M, eds. Fatty acid oxidation: Clinical, biochemical, and molecular aspects : proceedings of the International Symposium on Clinical, Biochemical, and Molecular Aspects of Fatty Oxidation held in Philadelphia, November 6-9, 1988. New York: Liss, 1990.

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5

Tomohito, Hamazaki, and Okuyama Harumi, eds. Fatty acids and lipids: New findings. Basel: Karger, 2001.

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6

Murphy, Elaine, Yann Nadjar, and Christine Vianey-Saban. Fatty Acid Oxidation, Electron Transfer and Riboflavin Metabolism Defects. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199972135.003.0008.

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The fatty acid oxidation disorders are a group of autosomally recessively inherited disorders of energy metabolism that may present with life-threatening hypoketotic hypoglycemia, encephalopathy and hepatic dysfunction, muscle symptoms, and/or cardiomyopathy. Milder phenotypes may present in adulthood, causing exercise intolerance, episodic rhabdomyolysis, and neuropathy. Specific investigations include acylcarnitine profiling, urine organic acid analysis, fibroblast or leucocyte studies of fatty acid oxidation flux/enzyme activity, and genetic testing. Management varies depending on the condition but includes avoidance of precipitants such as fasting, fever, and intense exercise, a high-carbohydrate, low-fat diet, and supplementation with carnitine or riboflavin. Inborn errors of riboflavin transport mainly present with Brown-Vialetto-Van Laere syndrome. Some patients respond dramatically to riboflavin supplementation; therefore it has to be tried in all suspected patients.
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7

Forrest, Rosemary, and Nicole Baugh. Genetic Mistakes: Understanding and Living with Fatty Acid Oxidation Disorders. Nova Science Publishers, Incorporated, 2017.

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8

Casteels, M. Differences in the Peroxisomal B-oxidation of Fatty Acids and Bile Acid Intermediates. Leuven University Press, 1990.

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9

Institution, British Standards, and European Committee for Standardization., eds. Fat and oil derivatives - Fatty Acid Methyl Esters (FAME): Determination of oxidation stability (accelerated oxidation test). BSI, 2003.

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10

Tanaka, Kay. Fatty Acid Oxidation: Clinical, Biochemical, and Molecular Aspects (Progress in Clinical and Biological Research). Wiley-Liss, 1990.

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11

Simon, Eaton, and Fatty Acid Oxidation and Ketogenesis Conference (4th : 1998 : London, England), eds. Current views of fatty acid oxidation and ketogenesis: From organelles to point mutations. New York: Kluwer Academic/Plenum Publishers, 1999.

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12

Simon, Eaton, and Fatty Acid Oxidation and Ketogenesis Conference (4th : 1998 : London, England), eds. Current views of fatty acid oxidation and ketogenesis: From organelles to point mutations. New York: Kluwer Academic/Plenum Publishers, 1999.

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13

Simon, Eaton, and Fatty Acid Oxidation and Ketogenesis Conference (4th : 1998 : London, England), eds. Current views of fatty acid oxidation and ketogenesis: From organelles to point mutations. New York: Kluwer Academic/Plenum Publishers, 1999.

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14

Fatty acid oxidation: Clinical biochemical, and molecular aspects : Proceedings of the International Symposium on Clinical, Biochemical and Molecular Aspects ... in clinical and biological research). Liss, 1990.

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15

Sarathy, Subram Maniam. Using an opposed flow diffusion flame to study the oxidation of C4 fatty acid methyl esters. 2006.

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16

Free fatty acid oxidation in obese and non-obese females and its response to aerobic exercise training. 1986.

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17

Duran, Marinus, and Isabel Tavares de Almeida. Interpretation of Acylcarnitine Analysis Results. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199972135.003.0085.

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The analysis of acylcarnitines in plasma or blood spot samples by tandem mass spectrometry will detect all 15 defects of mitochondrial fatty acid beta-oxidation, although false negative results may occur in well-fed, non-fasting patients. Moreover, more than 20 organic acidemias can be detected by this methodological approach. An acylcarnitine profile should be part of the work-up of patients presenting with rhabdomyolysis and/or hypoglycemia and adults with an unexplained leukoencephalopathy. Cases with abnormal acylcarnitines require an analysis of urine organic acids as well as enzyme activity evaluation and molecular investigations to confirm the inherited defect.
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18

(Editor), Patti A. Quant, and Simon Eaton (Editor), eds. Current Views of Fatty Acid Oxidation and Ketogenesis - From Organelles to Point Mutations (ADVANCES IN EXPERIMENTAL MEDICINE AND BIOLOGY Volume 466) (Advances in Experimental Medicine and Biology). Springer, 1999.

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19

Elliott, Perry, and Giuseppe Limongelli. Cardiac Aspects of INHERITED METABOLIC DISEASES. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199972135.003.0070.

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More than 40 inherited metabolic disorders cause heart disease, including fatty acid oxidation defects, glycogen storage disorders, lysosomal storage disorders, peroxisomal diseases, mitochondrial cytopathies, organic acidemias, aminoacidopathies, and congenital disorders of glycosylation. The pattern and severity of cardiac involvement varies between disorders but includes congenital heart diseases, heart muscle diseases, arrhythmias and sudden death, and heart failure. The majority of IMDs are multisystem diseases, but in a few cases cardiac disease is the predominant clinical feature and the main determinant of prognosis. For an increasing number of IEMs there are specific therapies designed to treat or ameliorate the effects of the underlying metabolic defect. In some cases, these therapies have an important effect on the progression of cardiac disease.
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20

Nys, Katelijne De. Peroxisomal B-Oxidation of Unsaturated Fatty Acids. Leuven Univ Pr, 2002.

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21

Vanhove, G. Peroxismal B-oxidation of 2-methyl-substituted Fatty Acids. Leuven University Press, 1994.

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22

Reddy, Ravinder M. D. Fatty Acids and Oxidative Stress in Neuropsychiatric Disorders. Nova Science Pub Inc, 2007.

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23

Ahmed, Ahmed I., Sarah Aldhaheri, and Allison Bannick. Inherited Metabolic Diseases (IMDs) and Pregnancy. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780190667351.003.0030.

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Inherited metabolic diseases (IMDs) are rare genetic disorders: clinically heterogeneous, and they can present at any age. With the expanded newborn screening panels, many of the IMDs have been successfully screened. Early diagnosis and treatment of these conditions have led to improved neurological outcomes and overall survival of these individuals, and now many of them are reaching childbearing age. Despite treatment, the potential presence of preexisting organ involvement may not only impact their fertility potentials but also may impose a higher risk of adverse maternal and fetal outcomes. Pregnancy leads to an extra strain on maternal metabolism; this may result in the manifestation of symptoms of a previously unknown disease or a progression of a known disease. This chapter will address the possible complications of some inherited disorders of metabolism that are associated with maternal or fetal neurological manifestations such as disorders of energy metabolism (eg, mitochondrial disorders, adult onset urea cycle disorders, ornithine transcarbamylase (OTC) deficiency, amino acidopathies, phenylketonuria (PKU), and impaired fatty acid oxidation disorders). We will provide special emphasis on the available potential treatments and plan of care during pregnancy and postpartum periods.
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24

Croes, K. X-Oxidation of 3-Methyl-Branched Fatty Acids - a Revised Pathway. Leuven University Press, 1998.

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25

Claudio, Galli, Simopoulos Artemis P. 1933-, Tremoli Elena, and International Society for the Study of Fatty Acids and Lipids., eds. Fatty acids and lipids: Biological aspects. Basel: Karger, 1994.

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26

-S, Chan H. W., ed. Autoxidation of unsaturated lipids. London: Academic Press, 1986.

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27

-S, Chan H. W., ed. Autoxidation of unsaturated lipids. London: Academic Press, 1987.

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28

Ljiljana Kostadinović, Sanja Popović, Dušica Čolović, Đ. Vukmirović, Tatjana Tasić, N. Puvača, and Jovanka Lević. Effect of extruded flaxseed in broiler diets on blood oxidative stability and meat fatty acid composition. Verlag Eugen Ulmer, 2016. http://dx.doi.org/10.1399/eps.2016.140.

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29

Human Longevity: Omega-3 Fatty Acids, Bioenergetics, Molecular Biology and Evolution. Taylor & Francis Group, 2014.

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30

Foulon, Veerie. Oxidation of 3-Methly-Branched Fatty Acids: Study of the Enzymes Involved in the Reaction Sequence (Acta Biomedica Lovaniensia, 235). Leuven Univ Pr, 2001.

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31

Hyde, Parker, Vincent J. Miller, and Jeff S. Volek. Keto-Adaptation in Health and Fitness. Edited by Dominic P. D’Agostino. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780190497996.003.0038.

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When dietary carbohydrate is restricted and protein consumed in moderation, the evolutionarily-conserved ketogenic metabolic machinery awakens. After just a few days circulating ketones increase by an order of magnitude, and over several weeks there is a profound shift away from glucose as the primary energy substrate to the preferred use of fatty acids and ketones. This metabolic process is known as keto-adaptation. The deemphasis on insulin-dependent glucose uptake into cells and concomitant increase in fat oxidation has important implications in management of insulin resistance and its secondary manifestations, which are all functionally carbohydrate-intolerant conditions. The health implications of keto-adaptation are profound. In a definitive break from traditional groupthink, athletes are now experimenting with diets low in carbohydrate in an effort to improve their health, body composition, performance, and recovery. This chapter explores the rationale for the construct of keto-adaptation as a tool for achieving general well-being and improved performance.
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32

Galli, Claudio, and Artemis P. Simopoulos. Fatty Acids and Lipids: Biological Aspects (World Review of Nutrition and Dietetics). S. Karger Publishers (USA), 1994.

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33

van Geel, Björn M., Marc Engelen, and Stephan Kemp. X-linked Adrenoleukodystrophy. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199972135.003.0061.

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X-linked adrenoleukodystrophy (X-ALD) is the most frequent peroxisomal disorder. Hallmarks are increased levels of plasma very long-chain fatty acids (VLCFA), mutations in the ABCD1 gene, impaired function of ALD-protein and, consequently, decreased import of VLCFA-CoA esters in peroxisomes and VLCFA beta-oxidation. Cerebral demyelination and axonal degeneration of the spinal cord are the main causes of neurological deficits. Endocrine dysfunction, particularly adrenocortical insufficiency, is very frequent. Based upon the age of onset of symptoms and the organs most severely affected, several phenotypes can be distinguished. Adrenomyeloneuropathy (AMN) and childhood cerebral adrenoleukodystrophy (CCALD) are the most frequent variants. At least 80% of female carriers will eventually develop neurological symptoms similar to men with AMN. The thin and scanty scalp hair in affected men may facilitate diagnosis of X-ALD. Identification of patients is of utmost importance, as adrenocortical insufficiency can be treated, rapidly progressive cerebral demyelination can be halted, and prenatal diagnostic testing is available. Furthermore, symptomatic therapies and multidisciplinary support may help patients coping with this disease.
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34

Lee, Ye-Sun. The effect of supplementation with n-9, n-6, and n-3 fatty acids on plasma lipid, lipoprotein, apolipoprotein B concentrations, LDL particle size, and oxidative susceptibility of two LDL subfractions in postmenopausal women. 1999.

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35

I. Giannenas, A. Tzora, E. Bonos, I. Sarakatsianos, A. Karamoutsios, I. Anastasiou, and I. Skoufos. Effects of dietary oregano essential oil, laurel essential oil and attapulgite on chemical composition, oxidative stability, fatty acid profile and mineral content of chicken breast and thigh meat. Verlag Eugen Ulmer, 2016. http://dx.doi.org/10.1399/eps.2016.134.

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