FEBS Letters | |
Secondary carnitine deficiency and impaired docosahexaenoic (22:6n‐3) acid synthesis: a common denominator in the pathophysiology of diseases of oxidative phosphorylation and β‐oxidation | |
Infante, Juan P1  Huszagh, Virginia A1  | |
[1] Institute for Theoretical Biochemistry and Molecular Biology, P.O. Box 4512, Ithaca, NY 14852, USA | |
关键词: Acyl-CoA dehydrogenase; Acylcarnitine; Cardiomyopathy; Adenine nucleotide translocator; Neuronal ceroid lipofuscinosis (Batten disease); Retinopathy; | |
DOI : 10.1016/S0014-5793(00)01083-8 | |
学科分类:生物化学/生物物理 | |
来源: John Wiley & Sons Ltd. | |
【 摘 要 】
A critical analysis of the literature of mitochondrial disorders reveals that genetic diseases of oxidative phosphorylation are often associated with impaired β-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 β-oxidation, producing secondary carnitine deficiency while increasing reactive oxygen species and depleting α-tocopherol (α-TOC). These events result in impairment of the recently elucidated mitochondrial pathway for synthesis of 22:6n-3-containing phospholipids, since carnitine and α-TOC are involved in their biosynthesis. Therapeutic supplementation with 22:6n-3 and α-TOC is suggested.
【 授权许可】
Unknown
【 预 览 】
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RO201912020309020ZK.pdf | 88KB | download |