Defects in mitochondrial β-oxidation of fatty acids

被引:15
|
作者
Wood, PA [1 ]
机构
[1] Univ Alabama, Sch Med, Dept Comparat Med, Birmingham, AL 35294 USA
[2] Univ Alabama, Sch Dent, Dept Comparat Med, Birmingham, AL 35294 USA
关键词
D O I
10.1097/00041433-199904000-00004
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Mitochondrial beta-oxidation of fatty acids generates energy by direct electron transfer at the dehydrogenase steps along with the ultimate product of acetyl-coenzyme A that can be further oxidized for ATP synthesis, or conversion to ketone bodies. This review describes the human inborn errors of this pathway and recent results concerning the development and use of mouse models of these inherited enzyme deficiencies. Curr Opin Lipidol 10:107-112. (C) 1999 Lippincott Williams & Wilkins.
引用
收藏
页码:107 / 112
页数:6
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