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Protein Ontology report - 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
PR:000004686 -
http://purl.obolibrary.org/obo/PR_000004686
Protein Forms
Ontology Information
Show OBO stanza
PRO ID
PR:000004686
PRO name
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Synonyms
PRO-Short-label
:
EXACT:
BCKDHA
Other
:
EXACT:
BCKDE1A | BCKDH E1-alpha | branched-chain alpha-keto acid dehydrogenase E1 component alpha chain
Definition
"A protein that is a translation product of the human BCKDHA gene or a 1:1 ortholog thereof." [PRO:DNx]
PRO Category
gene
Parent
PR:000000001
protein
Terms by PRO Category
Organism-Independent
Organism-Specific
Category
Number of Terms
Category
Number of Terms
Gene
1
Organism-Gene
2
Sequence
0
Organism-Sequence
2
Modification
1
Organism-Modification
7
Term Hierarchy
Visualization
DAG:
OLS:
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Protein Forms
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Compact View
Category
PRO ID
Name
Short Name
Definition
Ann.
Has Annotation?
Comp.
In Complex?
gene
PR:000004686
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
BCKDHA
"A protein that is a translation product of the human BCKDHA gene or a 1:1 ortholog thereof." [PRO:DNx]
organism-gene
PR:O45924
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (worm)
BCKDHA (worm)
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial that is encoded in the genome of worm." [PRO:DNx]
organism-gene
PR:P12694
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human)
hBCKDHA
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial that is encoded in the genome of human." [PRO:DNx, UniProtKB:P12694]
organism-sequence
PR:P12694-1
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform h1 (human)
hBCKDHA/iso:h1
"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]
organism-sequence
PR:P12694-2
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform h2 (human)
hBCKDHA/iso:h2
"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]
organism-modification
PR:000046471
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial, transit peptide removed form (human)
hBCKDHA/TransPep-
"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]
organism-modification
PR:000085806
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial sequence variant T310R, transit peptide removed form (human)
hBCKDHA/var:T310R/TransPep-
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that has an Arg residue at the position equivalent to Thr-310 of the amino acid sequence represented by UniProtKB:P12694-1 and that has had the transit peptide removed. Example: UniProtKB:P12694-1, 46-445, Thr-310, CHEBI:29952." [PRO:DNx, Reactome:R-HSA-9865052]
organism-modification
PR:000085873
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial sequence variant Y413C, transit peptide removed form (human)
hBCKDHA/var:Y413C/TransPep-
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that has a Cys residue at the position equivalent to Tyr-413 of the amino acid sequence represented by UniProtKB:P12694-1 and that has had the transit peptide removed. Example: UniProtKB:P12694-1, 46-445, Tyr-413, CHEBI:29950." [PRO:DNx, Reactome:R-HSA-9865065]
organism-modification
PR:000085894
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial sequence variant G290N, transit peptide removed form (human)
hBCKDHA/var:G290N/TransPep-
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that has an Asn residue at the position equivalent to Gly-290 of the amino acid sequence represented by UniProtKB:P12694-1 and that has had the transit peptide removed. Example: UniProtKB:P12694-1, 46-445, Gly-290, CHEBI:50347." [PRO:DNx, Reactome:R-HSA-9865040]
organism-modification
PR:000085912
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial sequence variant Y438N, transit peptide removed form (human)
hBCKDHA/var:Y438N/TransPep-
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that has an Asn residue at the position equivalent to Tyr-438 of the amino acid sequence represented by UniProtKB:P12694-1 and that has had the transit peptide removed. Example: UniProtKB:P12694-1, 46-445, Tyr-438, CHEBI:50347." [PRO:DNx, Reactome:R-HSA-9865058]
organism-modification
PR:000085929
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial sequence variant A285P, transit peptide removed form (human)
hBCKDHA/var:A285P/TransPep-
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that has a Pro residue at the position equivalent to Ala-285 of the amino acid sequence represented by UniProtKB:P12694-1 and that has had the transit peptide removed. Example: UniProtKB:P12694-1, 46-445, Ala-285, CHEBI:50342." [PRO:DNx, Reactome:R-HSA-9865056]
organism-modification
PR:000085940
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial sequence variant G249S, transit peptide removed form (human)
hBCKDHA/var:G249S/TransPep-
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that has a Ser residue at the position equivalent to Gly-249 of the amino acid sequence represented by UniProtKB:P12694-1 and that has had the transit peptide removed. Example: UniProtKB:P12694-1, 46-445, Gly-249, CHEBI:29999." [PRO:DNx, Reactome:R-HSA-9865067]
modification
PR:000019127
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial, transit peptide removed form
BCKDHA/TransPep-
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial that has had the transit peptide removed." [PRO:DNx]
organism-modification
PR:000046471
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial, transit peptide removed form (human)
hBCKDHA/TransPep-
"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]
organism-modification
PR:000085806
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial sequence variant T310R, transit peptide removed form (human)
hBCKDHA/var:T310R/TransPep-
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that has an Arg residue at the position equivalent to Thr-310 of the amino acid sequence represented by UniProtKB:P12694-1 and that has had the transit peptide removed. Example: UniProtKB:P12694-1, 46-445, Thr-310, CHEBI:29952." [PRO:DNx, Reactome:R-HSA-9865052]
organism-modification
PR:000085873
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial sequence variant Y413C, transit peptide removed form (human)
hBCKDHA/var:Y413C/TransPep-
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that has a Cys residue at the position equivalent to Tyr-413 of the amino acid sequence represented by UniProtKB:P12694-1 and that has had the transit peptide removed. Example: UniProtKB:P12694-1, 46-445, Tyr-413, CHEBI:29950." [PRO:DNx, Reactome:R-HSA-9865065]
organism-modification
PR:000085894
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial sequence variant G290N, transit peptide removed form (human)
hBCKDHA/var:G290N/TransPep-
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that has an Asn residue at the position equivalent to Gly-290 of the amino acid sequence represented by UniProtKB:P12694-1 and that has had the transit peptide removed. Example: UniProtKB:P12694-1, 46-445, Gly-290, CHEBI:50347." [PRO:DNx, Reactome:R-HSA-9865040]
organism-modification
PR:000085912
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial sequence variant Y438N, transit peptide removed form (human)
hBCKDHA/var:Y438N/TransPep-
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that has an Asn residue at the position equivalent to Tyr-438 of the amino acid sequence represented by UniProtKB:P12694-1 and that has had the transit peptide removed. Example: UniProtKB:P12694-1, 46-445, Tyr-438, CHEBI:50347." [PRO:DNx, Reactome:R-HSA-9865058]
organism-modification
PR:000085929
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial sequence variant A285P, transit peptide removed form (human)
hBCKDHA/var:A285P/TransPep-
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that has a Pro residue at the position equivalent to Ala-285 of the amino acid sequence represented by UniProtKB:P12694-1 and that has had the transit peptide removed. Example: UniProtKB:P12694-1, 46-445, Ala-285, CHEBI:50342." [PRO:DNx, Reactome:R-HSA-9865056]
organism-modification
PR:000085940
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial sequence variant G249S, transit peptide removed form (human)
hBCKDHA/var:G249S/TransPep-
"A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (human) that has a Ser residue at the position equivalent to Gly-249 of the amino acid sequence represented by UniProtKB:P12694-1 and that has had the transit peptide removed. Example: UniProtKB:P12694-1, 46-445, Gly-249, CHEBI:29999." [PRO:DNx, Reactome:R-HSA-9865067]
