Journal
FEBS LETTERS
Volume 468, Issue 1, Pages 1-5Publisher
WILEY
DOI: 10.1016/S0014-5793(00)01083-8
Keywords
acyl-CoA dehydrogenase; acylcarnitine; cardiomyopathy; adenine nucleotide translocator; neuronal ceroid lipofuscinosis (Batten disease); retinopathy
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A critical analysis of the literature of mitochondrial disorders reveals that genetic diseases of oxidative phosphorylation are often associated with impaired beta-oxidation, and vice versa, and preferentially affect brain, retina, heart and skeletal muscle, tissues which depend on docosahexaenoic (22:6n-3)containing phospholipids for functionality. Evidence suggests that an increased NADH/NAD(+) ratio generated by reduced flux through the respiratory chain inhibits beta-oxidation, producing secondary carnitine deficiency while increasing reactive oxygen species and depleting alpha-tocopherol (alpha-TOC). These events result in impairment of the recently elucidated mitochondrial pathway for synthesis of 22:6n-3-containing phospholipids, since carnitine and alpha-TOC are involved in their biosynthesis. Therapeutic supplementation with 22:6n-3 and alpha-TOC is suggested. (C) 2000 Federation of European Biochemical Societies.
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