InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: PCCA

Summary

InnateDB Protein

IDBP-46953.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

PCCA

Protein Name

propionyl CoA carboxylase, alpha polypeptide

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000365462

InnateDB Gene

IDBG-46949 (PCCA)

Protein Structure

UniProt Annotation

Function

Subcellular Localization

Mitochondrion matrix {ECO:0000269PubMed:16023992}.

Disease Associations

Propionic acidemia type I (PA-1) [MIM:606054]: Life- threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein. {ECO:0000269PubMed:10101253, ECO:0000269PubMed:10329019, ECO:0000269PubMed:12559849, ECO:0000269PubMed:15059621}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 8 experimentally validated interaction(s) in this database.

Experimentally validated
Total	8 [view]
Protein-Protein	8 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003824	catalytic activity
GO:0004075	biotin carboxylase activity
GO:0004658	propionyl-CoA carboxylase activity
GO:0005524	ATP binding
GO:0008716	D-alanine-D-alanine ligase activity
GO:0009374	biotin binding
GO:0016874	ligase activity
GO:0019899	enzyme binding
GO:0046872	metal ion binding

Biological Process

GO:0006635	fatty acid beta-oxidation
GO:0006766	vitamin metabolic process
GO:0006767	water-soluble vitamin metabolic process
GO:0006768	biotin metabolic process
GO:0008152	metabolic process
GO:0019626	short-chain fatty acid catabolic process
GO:0044255	cellular lipid metabolic process
GO:0044281	small molecule metabolic process

Cellular Component

GO:0005739	mitochondrion
GO:0005759	mitochondrial matrix
GO:0005829	cytosol

Protein Structure and Domains

PDB ID

InterPro

IPR000089 Biotin/lipoyl attachment
IPR003135 ATP-grasp fold, ATP-dependent carboxylate-amine ligase-type
IPR005479 Carbamoyl-phosphate synthetase large subunit-like, ATP-binding domain
IPR005481 Carbamoyl-phosphate synthase, large subunit, N-terminal
IPR005482 Biotin carboxylase, C-terminal
IPR011053 Single hybrid motif
IPR011054 Rudiment single hybrid motif
IPR011095 D-alanine--D-alanine ligase, C-terminal
IPR011761 ATP-grasp fold
IPR011764 Biotin carboxylation domain
IPR013651 ATP-grasp fold, RimK-type
IPR016185 Pre-ATP-grasp domain

PFAM