2024年12月24日 · DBT (Dihydrolipoamide Branched Chain Transacylase E2) is a Protein Coding gene. Diseases associated with DBT include Maple Syrup Urine Disease, Type Ii and Maple …

The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, …

The DBT gene provides instructions for making part of a group of enzymes called the branched-chain alpha-keto acid dehydrogenase (BCKD) enzyme complex. Learn about this gene and …

支链α-酮酸脱氢酶复合物 (BCKD) 是一种线粒体内酶复合物，参与支链氨基酸异亮氨酸、亮氨酸和缬氨酸的分解。 BCKD 复合体被认为由 24 个转酰基酶 (E2) 亚基以及相关的脱羧酶 (E1) 、脱 …

2016年11月3日 · Examination of the deletion mutation in the E2 (DBT) gene facilitated early MSUD diagnosis and was beneficial for the determination of the proper course of treatment.

2009年9月3日 · The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is catalyzed by the branched-chain alpha-keto acid …

Gene ID: 1629, updated on 8-Feb-2025. The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the …

2024年12月10日 · The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino …

This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their …

DBT - dihydrolipoamide branched chain transacylase E2 Synonym(s): BCKAD-E2, BCKDH-E2, BCOADC-E2, branched chain 2-oxo-acid dehydrogenase complex component E2, …