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BCKDHB 产品

(Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB))

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Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform. [provided by RefSeq, Jul 2008].

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Featured BCKDHB Categories

BCKDHB 抗体

High quality antibodies with extensive validation data.

BCKDHB 蛋白

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

Recommended BCKDHB 抗体

Product
Reactivity
Application
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Application WB, FACS, IF, IHC (p)
Validations
  • (6)
Cat. No. ABIN654629
Quantity 400 μL
Datasheet Datasheet
Reactivity Human
Application WB, FACS, IF, IHC (p), EIA
Validations
  • (5)
Cat. No. ABIN950620
Quantity 0.4 mL
Datasheet Datasheet
Reactivity Human
Application WB
Validations
  • (1)
Cat. No. ABIN6137489
Quantity 100 μL
Datasheet Datasheet

Recommended BCKDHB ELISA试剂盒

Product
Reactivity
Analytical Method
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Analytical Method Quantitative Sandwich ELISA
Validations
Cat. No. ABIN6209469
Quantity 96 tests
Datasheet Datasheet

Recommended BCKDHB 蛋白

Product
Reactivity
Source
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Source Wheat germ
Validations
  • (1)
Cat. No. ABIN1346529
Quantity 10 μg
Datasheet Datasheet
Reactivity Human
Source HEK-293 Cells
Validations
  • (1)
Cat. No. ABIN2715188
Quantity 20 μg
Datasheet Datasheet
Reactivity Rat
Source Yeast
Validations
Cat. No. ABIN1474395
Quantity 1 mg
Datasheet Datasheet

Synonyms and alternative names related to BCKDHB

branched chain keto acid dehydrogenase E1, beta polypeptide (BCKDHB), branched chain keto acid dehydrogenase E1 subunit beta (BCKDHB), branched chain keto acid dehydrogenase E1, beta polypeptide (bckdhb), branched chain keto acid dehydrogenase E1, beta polypeptide S homeolog (bckdhb.S), branched chain keto acid dehydrogenase E1 subunit beta (Bckdhb), branched chain ketoacid dehydrogenase E1, beta polypeptide (Bckdhb), BCKDHB, dJ279A18.1, E1B, zgc:158777

Protein level used designations for BCKDHB

  • 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
  • branched chain keto acid dehydrogenase E1, beta polypeptide (maple syrup urine disease)
  • mitochondrial branched-chain alpha-keto acid dehydrogenase E1-beta subunit
  • branched chain keto acid dehydrogenase E1, beta polypeptide
  • branched chain keto acid dehydrogenase E1 beta polypeptide
  • branched chain keto acid dehydrogenase E1 beta-like
  • 2-oxoisovalerate dehydrogenase beta subunit
  • BCKDE1B
  • BCKDH E1-beta
  • branched chain keto acid dehydrogenase E1 beta
  • branched-chain alpha-keto acid dehydrogenase E1 component beta chain
  • E1b-beta subunit of the branched-chain complex
  • branched chain alpha-ketoacid dehydrogenase E1-beta subunit
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