单词	branched-chain 2-keto acid dehydrogenase
释义	branched-chain 2-keto acid dehydrogenase 支链2-酮酸脱氢酶：多种酶复体，包括2-酮异戊酸脱氢酶(硫辛酰胺)，二氢硫辛酰胺脱氢酶(NAD)和二氢硫辛酰胺转酰基酶，含焦磷酸硫胺、硫辛酸和黄素腺嘌呤二硫苷酸(FAD)等物并需以FAD及辅酶A为其辅因子，作用为促使亮氨酸、异亮氨酸和缬氨酸等支链氨酸氧化脱羧。缺乏酶复体中某一成分，将引起槭糖尿病；参见disease项下maple syrup urine disease
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