BCKDHA antibody [C1C3]

Cannot supply to this region.

SKU:: GTX111382
Additional Names:: branched chain keto acid dehydrogenase E1, alpha polypeptide , BCKDE1A , MSU , MSUD1 , OVD1A
Application:: WB, IHC-P, IF, ICC
Concentration:: 0.31 mg/ml
Physical State:: Liquid
Species Reactivity:: Human
Purification:: Affinity Purified
Storage Conditions:: -20[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., 2-8[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., -20[o]C/-70[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted.
Supplier:: Genetex
Host:: Rabbit
Reactivities:: Human, Mouse, Rat
Buffer:: PBS, 1% BSA, 20% Glycerol, 0.025% ProClin 300.
Immunogen:: Recombinant protein encompassing a sequence within the C-terminus region of human BCKDHA. The exact sequence is proprietary.
Clone:: C1C3
Uniprot:: P12694
Synonyms:: 2-oxoisovalerate dehydrogenase (lipoamide);2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial;BCKDE1A;BCKDH E1-alpha;branched chain keto acid dehydrogenase E1 alpha protein;branched chain keto acid dehydrogenase E1, alpha polypeptide;branched-chain alpha-keto acid dehydrogenase E1 component alpha chain;MSU;MSUD1;OVD1A
Extra Details:: The BCKDHA gene encodes the E1-alpha subunit of the branched-chain alpha-keto acid (BCAA) dehydrogenase complex (BCKD; EC 1.2.4.4), an inner-mitochondrial enzyme complex that catalyzes the oxidative decarboxylation of the branched-chain alpha-ketoacids derived from isoleucine, leucine, and valine. This reaction is the second major step in the catabolism of the branched-chain amino acids (Wynn et al., 1998 [PubMed 9582350]). The BCKD complex consists of 3 catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a homo-24-meric dihydrolipoyl transacylase (E2; MIM 248610), and a homodimeric dihydrolipoamide dehydrogenase (E3; MIM 238331). E1 is a thiamine pyrophosphate (TPP)-dependent enzyme. The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The BCKDHB gene (MIM 248611) encodes the beta subunit of E1. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase.[supplied by OMIM]

Shipping Conditions:: Blue Ice