BCKDHB Products
(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 Antibodies
High quality antibodies with extensive validation data.
BCKDHB Proteins
Proteins for various applications incl. WB, ELISA, IF etc.
Recommended BCKDHB Antibodies
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 Kits
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 Proteins
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:158777Protein 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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