BCKDHB Products

Description

Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, andfunctions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components:branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease(MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physicalretardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different3' non-coding regions, but encoding the same isoform. (provided by RefSeq)

Bioinformatics

Product By Gene ID	594
Alternate Names	2-oxoisovalerate dehydrogenase beta subunit 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial BCKDE1B BCKDH E1-beta branched chain alpha-ketoacid dehydrogenase E1-beta subunit branched chain keto acid dehydrogenase E1, beta polypeptide Branched-chain alpha-keto acid dehydrogenase E1 component beta chain dJ279A18.1 E1B E1b-beta subunit of the branched-chain complex EC 1.2.4.4 FLJ17880

Research Areas for BCKDHB

Find related products by research area and learn more about each of the different research areas below.

Amino Acids, Drugs and other small molecules
Cancer
Signal Transduction