InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: DNAJC19

Summary

InnateDB Protein

IDBP-66680.5

Last Modified

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

Gene Symbol

DNAJC19

Protein Name

DnaJ (Hsp40) homolog, subfamily C, member 19

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000372005

InnateDB Gene

IDBG-66678 (DNAJC19)

Protein Structure

UniProt Annotation

Function

Probable component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. May act as a co-chaperone that stimulate the ATP-dependent activity (By similarity). {ECO:0000250}.

Subcellular Localization

Mitochondrion inner membrane {ECO:0000305PubMed:12592411}; Single-pass membrane protein {ECO:0000305PubMed:12592411}.

Disease Associations

3-methylglutaconic aciduria 5 (MGA5) [MIM:610198]: An autosomal recessive disorder characterized by early-onset dilated cardiomyopathy, growth failure, cerebellar ataxia causing significant motor delays, testicular dysgenesis, growth failure and significant increases in urine organic acids, particularly 3- methylglutaconic acid and 3-methylglutaric acid. {ECO:0000269PubMed:16055927}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Ubiquitously expressed.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 10 experimentally validated interaction(s) in this database.

Experimentally validated
Total	10 [view]
Protein-Protein	10 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession

GO Term

Biological Process

GO:0006457	protein folding
GO:0006626	protein targeting to mitochondrion
GO:0007601	visual perception
GO:0044267	cellular protein metabolic process
GO:0048806	genitalia development