BCKDHB Antibody (C-term) Blocking peptide

Background:
Branched-chain keto acid dehydrogenase is a multienzymecomplex associated with the inner membrane of mitochondria, andfunctions in the catabolism of branched-chain amino acids. Thecomplex consists of multiple copies of 3 components: branched-chainalpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2)and lipoamide dehydrogenase (E3). This gene encodes the E1 betasubunit, and mutations therein have been associated with maplesyrup urine disease (MSUD), type 1B, a disease characterized by amaple syrup odor to the urine in addition to mental and physicalretardation, and feeding problems.

Other Names:
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain, BCKDE1B, BCKDH E1-beta, BCKDHB

Gene Name: BCKDHB (HGNC:987)

Gene ID: 594
Primary Accession: P21953
Other Accession: NP_000047.1
Format: Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed.