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Merck

BCKDHB

branched chain keto acid dehydrogenase E1, beta polypeptide

Synonyms:
E1B, dJ279A18.1
Species:
Human BCKDHB (594), Mouse Bckdhb (12040), Rat Bckdhb (29711), cow BCKDHB (282150), domestic cat BCKDHB (101080635), dog BCKDHB (474978), chicken BCKDHB (395375), Zebrafish bckdhb (569980), domestic guinea pig Bckdhb (100731951), naked mole-rat Bckdhb (101725476) ...More
UniProtKB ID:
P21953, Q9BQL0
Gene ID:
  • Human(594) Summary: 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]
  • Mouse(12040) branched chain ketoacid dehydrogenase E1, beta polypeptide
  • Rat(29711) branched chain keto acid dehydrogenase E1, beta polypeptide
  • cow(282150) branched chain keto acid dehydrogenase E1, beta polypeptide
  • domestic cat(101080635) branched chain keto acid dehydrogenase E1, beta polypeptide
  • dog(474978) branched chain keto acid dehydrogenase E1, beta polypeptide
  • chicken(395375) branched chain keto acid dehydrogenase E1, beta polypeptide
  • Zebrafish(569980) branched chain ketoacid dehydrogenase E1, beta polypeptide
  • domestic guinea pig(100731951) branched chain keto acid dehydrogenase E1, beta polypeptide
  • naked mole-rat(101725476) branched chain keto acid dehydrogenase E1, beta polypeptide
  • Domestic Rabbit(100353682) branched chain keto acid dehydrogenase E1, beta polypeptide
  • sheep(101113939) branched chain keto acid dehydrogenase E1, beta polypeptide
  • Horse(100065300) branched chain keto acid dehydrogenase E1, beta polypeptide

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Antibodies

Product Number
Description
Species Reactivity
Application
HPA031956
Anti-BCKDHB antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution,
Species Reactivity
human
Application
immunofluorescence
SAB1302827
ANTI-BCKDHB (N-TERM) antibody produced in rabbit, IgG fraction of antiserum, buffered aqueous solution,
Species Reactivity
human
Application
flow cytometry
immunofluorescence
immunohistochemistry
western blot