BCKDHA
branched chain keto acid dehydrogenase E1, alpha polypeptide
Sinonimi:
BCKDE1A, MSU, MSUD1, OVD1A
Specie:
Human BCKDHA (593), Mouse Bckdha (12039), Rat Bckdha (25244), Zebrafish bckdha (554124), chicken BCKDHA (374210), dog BCKDHA (484488), Horse BCKDHA (100068678), cow BCKDHA (282149), domestic cat BCKDHA (101087186), sheep BCKDHA (100145857) ...Più
ID UniProtKB:
ID gene:
- Human(593) Summary: 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]
- Mouse(12039) branched chain ketoacid dehydrogenase E1, alpha polypeptide
- Rat(25244) branched chain ketoacid dehydrogenase E1, alpha polypeptide
- Zebrafish(554124) branched chain keto acid dehydrogenase E1, alpha polypeptide
- chicken(374210) branched chain keto acid dehydrogenase E1, alpha polypeptide
- dog(484488) branched chain keto acid dehydrogenase E1, alpha polypeptide
- Horse(100068678) branched chain keto acid dehydrogenase E1, alpha polypeptide
- cow(282149) branched chain keto acid dehydrogenase E1, alpha polypeptide
- domestic cat(101087186) branched chain keto acid dehydrogenase E1, alpha polypeptide
- sheep(100145857) branched chain keto acid dehydrogenase E1, alpha polypeptide
- Domestic Rabbit(100352048) branched chain keto acid dehydrogenase E1, alpha polypeptide
- domestic guinea pig(100715185) branched chain keto acid dehydrogenase E1, alpha polypeptide
- naked mole-rat(101705928) branched chain keto acid dehydrogenase E1, alpha polypeptide
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Antibodies
N° Catalogo
Descrizione
Reattività con
Applicazione
Anti-BCKDHA antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution,
Reattività con
human
Applicazione
immunohistochemistry
Anti-BCKDHA, (N-terminal) antibody produced in rabbit, affinity isolated antibody,
Reattività con
bovine, dog, guinea pig, horse, human, mouse, rabbit, rat, sheep
Applicazione
immunohistochemistry
western blot
western blot
Anti-BCKDHA antibody produced in mouse, purified immunoglobulin, buffered aqueous solution,
Reattività con
human
Applicazione
western blot
esiRNA
N° Catalogo
Descrizione
Specie