Entity Details

Primary name PCCA_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP05165
EntryNamePCCA_HUMAN
FullNamePropionyl-CoA carboxylase alpha chain, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length728
SequenceStatuscomplete
DateCreated1987-08-13
DateModified2021-06-02

Ontological Relatives

GenesPCCA

GO terms

Show/Hide Table
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:0009374 biotin binding
GO:0016421 CoA carboxylase activity
GO:0019626 short-chain fatty acid catabolic process
GO:0019899 enzyme binding
GO:0046872 metal ion binding

Subcellular Location

Show/Hide Table
Subcellular Location
Mitochondrion matrix

Domains

Show/Hide Table
DomainNameCategoryType
IPR000089 Biotin/lipoyl attachmentDomainDomain
IPR001882 Biotin-binding siteSiteBinding site
IPR005479 Carbamoyl-phosphate synthetase large subunit-like, ATP-binding domainDomainDomain
IPR005481 Biotin carboxylase-like, N-terminal domainDomainDomain
IPR005482 Biotin carboxylase, C-terminalDomainDomain
IPR011053 Single hybrid motifFamilyHomologous superfamily
IPR011054 Rudiment single hybrid motifFamilyHomologous superfamily
IPR011761 ATP-grasp foldDomainDomain
IPR011764 Biotin carboxylation domainDomainDomain
IPR016185 Pre-ATP-grasp domain superfamilyFamilyHomologous superfamily
IPR041265 Propionyl-coenzyme A carboxylase, BT domainDomainDomain

Diseases

Show/Hide Table
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

Show/Hide Table
DrugNameSourceType
DB00121 BiotinDrugbanksmall molecule