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BCKDHA Products

(Branched Chain Keto Acid Dehydrogenase E1, alpha Polypeptide (BCKDHA))

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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].

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Featured BCKDHA Categories

BCKDHA Antibodies

High quality antibodies with extensive validation data.

BCKDHA ELISA Kits

Reliable ELISA kits for a wide range of species.

BCKDHA Proteins

Proteins for various applications incl. WB, ELISA, IF etc.

Recommended BCKDHA Antibodies

Product
Reactivity
Application
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Application WB, IHC (p), FACS
Validations
  • (3)
Cat. No. ABIN390811
Quantity 400 μL
Datasheet Datasheet
Reactivity Human
Application WB, ELISA, IHC
Validations
  • (2)
Cat. No. ABIN7142166
Quantity 100 μg
Datasheet Datasheet
Reactivity Human
Application WB, ELISA
Validations
  • (2)
Cat. No. ABIN560039
Quantity 50 μL
Datasheet Datasheet

Recommended BCKDHA ELISA Kits

Product
Reactivity
Analytical Method
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Analytical Method Quantitative Sandwich ELISA
Validations
  • (1)
Cat. No. ABIN5519933
Quantity 96 tests
Datasheet Datasheet
Reactivity Rat
Analytical Method
Validations
Cat. No. ABIN1118749
Quantity 96 tests
Datasheet Datasheet
Reactivity Human
Analytical Method
Validations
Cat. No. ABIN1118747
Quantity 96 tests
Datasheet Datasheet

Recommended BCKDHA Proteins

Product
Reactivity
Source
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Source Wheat germ
Validations
  • (1)
Cat. No. ABIN1346527
Quantity 10 μg
Datasheet Datasheet
Reactivity Human
Source Wheat germ
Validations
  • (1)
Cat. No. ABIN1346525
Quantity 10 μg
Datasheet Datasheet
Reactivity Human
Source Escherichia coli (E. coli)
Validations
  • (1)
Cat. No. ABIN7122029
Quantity 50 μg
Datasheet Datasheet

Latest Publications for our BCKDHA products

Zhou, Shao, Wu, Shu, Dong, Liu, Chen, Wynn, Wang, Wang, Gui, Qi, Lusis, Li, Wang, Ning, Yang, Chuang, Wang, Sun: "Targeting BCAA Catabolism to Treat Obesity-Associated Insulin Resistance." in: Diabetes, Vol. 68, Issue 9, pp. 1730-1746, (2019) (PubMed).

Wallace, Green, Roberts, Lee, McCarville, Sanchez-Gurmaches, Meurs, Gengatharan, Hover, Phillips, Ciaraldi, Guertin, Cabrales, Ayres, Nomura, Loomba, Metallo: "Enzyme promiscuity drives branched-chain fatty acid synthesis in adipose tissues." in: Nature chemical biology, Vol. 14, Issue 11, pp. 1021-1031, (2018) (PubMed).

Green, Wallace, Divakaruni, Phillips, Murphy, Ciaraldi, Metallo: "Branched-chain amino acid catabolism fuels adipocyte differentiation and lipogenesis." in: Nature chemical biology, Vol. 12, Issue 1, pp. 15-21, (2015) (PubMed).

Synonyms and alternative names related to BCKDHA

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (LOC704978), branched chain keto acid dehydrogenase E1, alpha polypeptide (bckdha), branched chain keto acid dehydrogenase E1, alpha polypeptide (BCKDHA), 2-oxoisovalerate dehydrogenase alpha subunit (branched-chain alpha-keto acid dehydrogenase E1 component alpha chain) (BCKDH E1-alpha) (BCKDE1A) (BckdhA), branched chain ketoacid dehydrogenase E1, alpha polypeptide (Bckdha), BCKDA, BCKDE1A, E1a, MSU, MSUD1, OVD1A, wu:fd20d04, zgc:110049

Protein level used designations for BCKDHA

  • branched chain keto acid dehydrogenase E1, alpha polypeptide
  • 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
  • 2-oxoisovalerate dehydrogenase alpha subunit (branched-chain alpha-keto acid dehydrogenase E1 component alpha chain) (BCKDH E1-alpha) (BCKDE1A)
  • 2-oxoisovalerate dehydrogenase (lipoamide)
  • BCKDH E1-alpha
  • branched-chain alpha-keto acid dehydrogenase E1 component alpha chain
  • branched chain keto acid dehydrogenase E1 alpha polypeptide
  • BCKDE1A
  • branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease)
  • BCKAD E1[a]
  • branched chain alpha-ketoacid dehydrogenase subunit E1 alpha
  • branched chain keto acid dehydrogenase subunit E1, alpha polypeptide
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