Bckdha
entrez
25244
canonical
uniprot
P11960
canonical
ensembl
ENSRNOG00000020607
resolver
ensp
ENSRNOP00000109641
resolver
genesymbol
Bckdha
resolver
uniprot
B1WBN3
resolver
uniprot
Q5EB89
resolver
uniprot_entry
B1WBN3_RAT
resolver
uniprot_entry
ODBA_RAT
resolver
uniprot_entry
Q5EB89_RAT
resolver
amino_acid_sequence
SAAKIWRPSRGLRQAALLLLGRPGARGLARFHPSRQQQQQFPSLDDKPQFPGASAEFVDKLEFIQPNVISGIPIYRVMDRQGQIINPSEDPHLPQEEVLKLYRSMTLLNTMDRILYESQRQGRISFYMTNYGEEGTHVGSAAALERTDLVFGQYREAGVLMYRDYPLELFMAQCYGNVSDPGKGRQMPVHYGCKERHFVTISSPLATQIPQAVGAAYAAKRANANQIVICYFGEGAASEGDAHAGFNFAATLECPIIFFCRNNGYAISTPTSEQYRGDGIAARGPGYGIMSIRVDGNDVFAVYNATKEARRRAVAENQPFLIEAMTYRIGHHSTSDDSSAYRSVDEVNYWDKQDHPISRLRQYLLNQGWWDEEQEKAWRKQSRKKVMEAFEQAERKLKPNPSLLFSDVYQEMPAQLRRQQESLARHLQTYGEHYPLDHFDK
uniprot
proteins
ec_number
1.2.4.4
uniprot
proteins
function
FUNCTION: Together with BCKDHB forms the heterotetrameric E1 subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex. The BCKD complex catalyzes the multi-step oxidative decarboxylation of alpha-ketoacids derived from the branched-chain amino-acids valine, leucine and isoleucine producing CO2 and acyl-CoA which is subsequently utilized to produce energy. The E1 subunit catalyzes the first step with the decarboxylation of the alpha-ketoacid forming an enzyme-product intermediate. A reductive acylation mediated by the lipoylamide cofactor of E2 extracts the acyl group from the E1 active site for the next step of the reaction. {ECO:0000250|UniProtKB:P12694}.
uniprot
proteins
mass_dalton
50164
uniprot
proteins
post_translational_modification
PTM: Phosphorylated at Ser-333 by BCKDK and dephosphorylated by protein phosphatase PPM1K. {ECO:0000269|PubMed:29779826}.
uniprot
proteins
protein_family
BCKDHA family
uniprot
proteins
pubmed
16396499
uniprot
proteins
pubmed
22673903
uniprot
proteins
pubmed
2822716
uniprot
proteins
pubmed
29779826
uniprot
proteins
sequence_length
441
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion matrix {ECO:0000250|UniProtKB:P12694}.
uniprot
proteins