[Term] id: PR:P21953-1 name: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform h1 (human) def: "A 2-oxoisovalerate dehydrogenase subunit beta, 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:P21953-1." [PRO:DNx, UniProtKB:P21953] comment: Category=organism-sequence. synonym: "hBCKDHB/iso:h1" EXACT PRO-short-label [PRO:DNx] xref: UniProtKB:P21953-1 is_a: PR:P21953 ! 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (human) relationship: only_in_taxon NCBITaxon:9606 ! Homo sapiens [Term] id: PR:000046472 name: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, transit peptide removed form (human) def: "A 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (human) that has had the transit peptide removed. UniProtKB:P21953, 51-392." [PRO:DNx, Reactome:R-HSA-70015] comment: Category=organism-modification. synonym: "PRO_0000020470" EXACT PRO-proteoform-ftid [PRO:DNx] synonym: "hBCKDHB/TransPep-" EXACT PRO-short-label [PRO:DNx] synonym: "UniProtKB:P21953, 51-392" EXACT PRO-proteoform-std [PRO:DNx] xref: Reactome:R-HSA-70015 "PR:000046472 located_in GO:0005759" intersection_of: PR:P21953 ! 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (human) intersection_of: lacks_part SO:0000725 ! transit_peptide relationship: only_in_taxon NCBITaxon:9606 ! Homo sapiens [Term] id: PR:P21953-2 name: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform h2 (human) def: "A 2-oxoisovalerate dehydrogenase subunit beta, 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:P21953-2." [PRO:DNx, UniProtKB:P21953] comment: Category=organism-sequence. synonym: "hBCKDHB/iso:h2" EXACT PRO-short-label [PRO:DNx] xref: UniProtKB:P21953-2 is_a: PR:P21953 ! 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (human) relationship: only_in_taxon NCBITaxon:9606 ! Homo sapiens [Term] id: PR:000004687 name: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial def: "A protein that is a translation product of the human BCKDHB gene or a 1:1 ortholog thereof." [PRO:DNx] comment: Category=gene. synonym: "BCKDHB" EXACT PRO-short-label [PRO:DNx] synonym: "BCKDE1B" EXACT [] synonym: "BCKDH E1-beta" EXACT [] synonym: "branched-chain alpha-keto acid dehydrogenase E1 component beta chain" EXACT [] is_a: PR:000000001 ! protein [Term] id: PR:P21953 name: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (human) def: "A 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial that is encoded in the genome of human." [PRO:DNx, UniProtKB:P21953] comment: Category=organism-gene. synonym: "BCKDHB" RELATED Gene-based [UniProtKB:P21953] synonym: "hBCKDHB" EXACT PRO-short-label [PRO:DNx] synonym: "BCKDE1B (human)" EXACT [UniProtKB:P21953] synonym: "BCKDH E1-beta (human)" EXACT [UniProtKB:P21953] synonym: "branched-chain alpha-keto acid dehydrogenase E1 component beta chain (human)" EXACT [UniProtKB:P21953] xref: UniProtKB:P21953 intersection_of: only_in_taxon NCBITaxon:9606 ! Homo sapiens intersection_of: PR:000004687 ! 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial relationship: has_gene_template HGNC:987 ! BCKDHB (human)