ACAD8 Q9UKU7

异丁酰辅酶A脱氢酶，线粒体 (IBDH) (EC 1.3.8.5) (激活剂招募辅因子42 kDa组分) (ARC42) (酰基辅酶A脱氢酶家族成员8) (ACAD-8)

蛋白质信息 (UniProt)

异丁酰辅酶A脱氢酶，线粒体 (IBDH) (EC 1.3.8.5) (激活剂招募辅因子42 kDa组分) (ARC42) (酰基辅酶A脱氢酶家族成员8) (ACAD-8)

Q9UKU7

功能描述

异丁酰-CoA脱氢酶，在缬氨酸分解代谢途径中催化2-甲基丙酰-CoA转化为(2E)-2-甲基丙烯酰-CoA (PubMed:11013134, PubMed:12359132, PubMed:16857760)。在较小程度上，也能够催化(2S)-2-甲基丁酰-CoA的氧化 (PubMed:11013134, PubMed:12359132)。 {ECO:0000269|PubMed:11013134, ECO:0000269|PubMed:12359132, ECO:0000269|PubMed:16857760}。

组织特异性

Detected at comparable levels in heart, lung, brain, skeletal muscle, pancreas and placenta. Weakly expressed in liver and kidney.

亚细胞定位

Mitochondrion

关键词

3D-structure Acetylation Activator Alternative splicing Branched-chain amino acid catabolism Direct protein sequencing Disease variant FAD Flavoprotein Mitochondrion

外部链接

UniProt: Q9UKU7

NCBI Gene

GeneCards