Entity Details

Primary name PCCB_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP05166
EntryNamePCCB_HUMAN
FullNamePropionyl-CoA carboxylase beta chain, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length539
SequenceStatuscomplete
DateCreated1987-08-13
DateModified2021-06-02

Ontological Relatives

GenesPCCB

GO terms

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GOName
GO:0004658 propionyl-CoA carboxylase activity
GO:0005524 ATP binding
GO:0005739 mitochondrion
GO:0005759 mitochondrial matrix
GO:0005829 cytosol
GO:0006768 biotin metabolic process
GO:0019626 short-chain fatty acid catabolic process

Subcellular Location

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Subcellular Location
Mitochondrion matrix

Domains

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DomainNameCategoryType
IPR011762 Acetyl-coenzyme A carboxyltransferase, N-terminalDomainDomain
IPR011763 Acetyl-coenzyme A carboxyltransferase, C-terminalDomainDomain
IPR029045 ClpP/crotonase-like domain superfamilyFamilyHomologous superfamily
IPR034733 Acetyl-CoA carboxylaseDomainDomain

Diseases

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Disease IDSourceNameDescription
606054 OMIMPropionic acidemia type I (PA-1)Life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB00121 BiotinDrugbanksmall molecule
DB00161 ValineDrugbanksmall molecule