[Term]
id: PR:P12694-2
name: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform h2 (human)
def: "A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that is a translation product of some mRNA whose exon structure and start site selection renders it capable of giving rise to a protein with the amino acid sequence represented by UniProtKB:P12694-2." [PRO:DNx, UniProtKB:P12694]
comment: Category=organism-sequence.
synonym: "hBCKDHA/iso:h2" EXACT PRO-short-label [PRO:DNx]
xref: UniProtKB:P12694-2
is_a: PR:P12694 ! 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human)
relationship: only_in_taxon NCBITaxon:9606 ! Homo sapiens

[Term]
id: PR:000046471
name: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial, transit peptide removed form (human)
def: "A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that has had the transit peptide removed. UniProtKB:P12694, 46-445." [PRO:DNx, Reactome:R-HSA-70014]
comment: Category=organism-modification.
synonym: "PRO_0000020465" EXACT PRO-proteoform-ftid [PRO:DNx]
synonym: "hBCKDHA/TransPep-" EXACT PRO-short-label [PRO:DNx]
synonym: "UniProtKB:P12694, 46-445" EXACT PRO-proteoform-std [PRO:DNx]
xref: Reactome:R-HSA-70014 "PR:000046471 located_in GO:0005759"
intersection_of: PR:P12694 ! 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human)
intersection_of: lacks_part SO:0000725 ! transit_peptide
relationship: only_in_taxon NCBITaxon:9606 ! Homo sapiens

[Term]
id: PR:P12694
name: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human)
def: "A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial that is encoded in the genome of human." [PRO:DNx, UniProtKB:P12694]
comment: Category=organism-gene.
synonym: "BCKDHA" RELATED Gene-based [UniProtKB:P12694]
synonym: "hBCKDHA" EXACT PRO-short-label [PRO:DNx]
synonym: "BCKDE1A (human)" EXACT [UniProtKB:P12694]
synonym: "BCKDH E1-alpha (human)" EXACT [UniProtKB:P12694]
synonym: "branched-chain alpha-keto acid dehydrogenase E1 component alpha chain (human)" EXACT [UniProtKB:P12694]
xref: UniProtKB:P12694
intersection_of: only_in_taxon NCBITaxon:9606 ! Homo sapiens
intersection_of: PR:000004686 ! 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
relationship: has_gene_template HGNC:986 ! BCKDHA (human)

[Term]
id: PR:000004686
name: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
def: "A protein that is a translation product of the human BCKDHA gene or a 1:1 ortholog thereof." [PRO:DNx]
comment: Category=gene.
synonym: "BCKDHA" EXACT PRO-short-label [PRO:DNx]
synonym: "BCKDE1A" EXACT []
synonym: "BCKDH E1-alpha" EXACT []
synonym: "branched-chain alpha-keto acid dehydrogenase E1 component alpha chain" EXACT []
is_a: PR:000000001 ! protein

[Term]
id: PR:P12694-1
name: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform h1 (human)
def: "A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that is a translation product of some mRNA whose exon structure and start site selection renders it capable of giving rise to a protein with the amino acid sequence represented by UniProtKB:P12694-1." [PRO:DNx, UniProtKB:P12694]
comment: Category=organism-sequence.
synonym: "hBCKDHA/iso:h1" EXACT PRO-short-label [PRO:DNx]
xref: UniProtKB:P12694-1
is_a: PR:P12694 ! 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human)
relationship: only_in_taxon NCBITaxon:9606 ! Homo sapiens