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BCKDHA / BCKDE1A

branched chain keto acid dehydrogenase E1, alpha polypeptide

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.

Gene Name: branched chain keto acid dehydrogenase E1, alpha polypeptide
Synonyms: BCKDHA, BCKDH E1-alpha, BCKDE1A, MSU, MSUD1, OVD1A
Target Sequences: NM_000709 NP_000700.1 P12694

Publications (1)

1
SIRT4 is an early regulator of branched-chain amino acid catabolism that promotes adipogenesis. Elma Zaganjor, Haejin Yoon, Jessica B Spinelli, Elizabeth R Nunn, Gaëlle Laurent, Paulina Keskinidis, Suganja Sivaloganathan, Shakchhi Joshi, Giulia Notarangelo, Stacy Mulei, Mathew T Chvasta, Sarah A Tucker, Krystle Kalafut, Robert A H van de Ven, Clary B Clish, Marcia C Haigis. Cell reports. 2021 July;36:109345. [Full Text Article] [PubMed:34260923] [PMC:PMC8320302]

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Proteins (5)
Over-Expression Lysate (2)
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BCKDHA / BCKDE1A (5)
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GST (1)
GST, N-terminus (1)
His (1)
Myc-DDK (Flag) (2)
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293T Cells (1)
E. coli (1)
HEK 293 Cells (1)
Wheat Germ Extract (2)
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BCKDHA / BCKDE1A Protein
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E. coli
His
10 µg/$311; 50 µg/$498; 20 µg/$386; 100 µg/$790; 250 µg/$1,278; 500 µg/$1,917
BCKDHA / BCKDE1A Protein - 12.5% SDS-PAGE Stained with Coomassie Blue.
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Wheat Germ Extract
GST, N-terminus
10 µg/$479; 25 µg/$670
BCKDHA / BCKDE1A Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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293T Cells
Myc-DDK (Flag)
50.4 kDa
20 µg/$150
BCKDHA / BCKDE1A Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
50.4 kDa
100 µg/$494
BCKDHA / BCKDE1A Protein - 12.5% SDS-PAGE of human BCKDHA stained with Coomassie Blue
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Wheat Germ Extract
GST
74.69 kDa
10 µg/$479; 25 µg/$670
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For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).