Automated description from the Alliance of Genome Resources (Release 8.3.0)
Predicted to enable metal ion binding activity and oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor. Predicted to contribute to branched-chain 2-oxo acid dehydrogenase activity. Predicted to be involved in branched-chain alpha-keto acid decarboxylation to branched-chain acyl-CoA. Located in mitochondrion. Is expressed in several structures, including alimentary system; cardiovascular system; central nervous system; genitourinary system; and sensory organ. Human ortholog(s) of this gene implicated in maple syrup urine disease and maple syrup urine disease type IA. Orthologous to human BCKDHA (branched chain keto acid dehydrogenase E1 subunit alpha).
This site uses cookies.
Some cookies are essential for site operations and others help us analyze use and utility of our web site.
Please refer to our
privacy policy
for more information.