Entity Details

Primary name ODPA_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP08559
EntryNameODPA_HUMAN
FullNamePyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length390
SequenceStatuscomplete
DateCreated1988-08-01
DateModified2021-06-02

Ontological Relatives

GenesPDHA1

GO terms

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GOName
GO:0004739 pyruvate dehydrogenase (acetyl-transferring) activity
GO:0005634 nucleus
GO:0005730 nucleolus
GO:0005739 mitochondrion
GO:0005759 mitochondrial matrix
GO:0005967 mitochondrial pyruvate dehydrogenase complex
GO:0006006 glucose metabolic process
GO:0006086 acetyl-CoA biosynthetic process from pyruvate
GO:0006090 pyruvate metabolic process
GO:0006099 tricarboxylic acid cycle
GO:0034604 pyruvate dehydrogenase (NAD+) activity
GO:0045254 pyruvate dehydrogenase complex
GO:0061732 mitochondrial acetyl-CoA biosynthetic process from pyruvate

Subcellular Location

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Subcellular Location
Mitochondrion matrix

Domains

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DomainNameCategoryType
IPR001017 Dehydrogenase, E1 componentDomainDomain
IPR017597 Pyruvate dehydrogenase (acetyl-transferring) E1 component, alpha subunit, subgroup yFamilyFamily
IPR029061 Thiamin diphosphate-binding foldFamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
312170 OMIMPyruvate dehydrogenase E1-alpha deficiency (PDHAD)An enzymatic defect causing primary lactic acidosis in children. It is associated with a broad clinical spectrum ranging from fatal lactic acidosis in the newborn to chronic neurologic dysfunction with structural abnormalities in the central nervous system without systemic acidosis. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB00157 NADHDrugbanksmall molecule