Mitochondrial and peroxisomal metabolism of glutaryl-CoA.
Mitochondrial and peroxisomal metabolism of glutaryl-CoA.
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戊二酰辅酶A的线粒体和过氧化物酶体代谢。
DOI：
10.1111/j.1432-1033.1985.tb08702.x
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发表时间：
1985
期刊：
影响因子：
--
通讯作者：
VanHoof,F
中科院分区：
文献类型：
--
作者：
Vamecq,J;deHoffmann,E;VanHoof,F
使用从肝脏过氧化物酶体中纯化的组分，我们证明戊二酰辅酶A氧化酶反应的产物是戊烯二酰辅酶A和H2 O2。在整个组织匀浆中，当FADH 2的再氧化被阻断时，戊二酰辅酶A脱氢酶对戊二酰辅酶A的处理被抑制。然而，在这些条件下，仍然可以发生戊烯二酰辅酶A脱羧，并且从标记的戊二酰辅酶A以摩尔/摩尔比与H2 O2产生14 CO2。这些数据表明，在不存在其线粒体脱氢的情况下，戊二酰辅酶A在过氧化物酶体中被氧化为戊烯二酰辅酶A，后者可能转移到线粒体，在线粒体中脱羧并进一步加工。戊二酸尿症I型和II型。
Using a fraction purified from liver peroxisomes, we demonstrate that products of the glutaryl‐CoA oxidase reaction are glutaconyl‐CoA and H2O2. No glutaconyl‐CoA decarboxylation occurs with this fraction.In whole tissue homogenates, the handling of glutaryl‐CoA by glutaryl‐CoA dehydrogenase is inhibited when reoxidation of FADH2is blocked. Under these conditions, glutaconyl‐CoA decarboxylation, however, can still occur and14CO2is produced from labelled glutaryl‐CoA in mole/mole ratio with H2O2.These data indicate that in the absence of its mitochondrial dehydrogenation, glutaryl‐CoA is oxidized in peroxisomes to glutaconyl‐CoA which is probably transferred to mitochondria where it is decarboxylated and further processed.This hypothesis allows coherent explanation for the observed organic aciduria in both glutaricaciduria types I and II.