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  2. Polyclonal

GTX45109

BCKDHA antibody, N-term

Cannot supply to this region.

SKU:
GTX45109
Additional Names:
branched chain keto acid dehydrogenase E1, alpha polypeptide , BCKDE1A , MSU , MSUD1 , OVD1A
Application:
WB, IHC-P
Concentration:
0.5-1 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
Buffer:
PBS, 2% Sucrose, 0.09% Sodium azide.
Immunogen:
A synthetic peptide corresponding to a N-terminal region of Human BCKDHA
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 branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Sep 2009]
Shipping Conditions:
Blue Ice