Aliases for DNAJC19 Gene
External Ids for DNAJC19 Gene
The protein encoded by this gene is thought to be part of a complex involved in the ATP-dependent transport of transit peptide-containing proteins from the inner cell membrane to the mitochondrial matrix. Defects in this gene are a cause of 3-methylglutaconic aciduria type 5 (MGA5), also known as dilated cardiomyopathy with ataxia (DCMA). Alternative splicing of this gene results in multiple transcript variants. Related pseudogenes have been identified on chromosomes 1, 2, 6, 10, 14 and 19. [provided by RefSeq, Jan 2012]
GeneCards Summary for DNAJC19 Gene
DNAJC19 (DnaJ (Hsp40) Homolog, Subfamily C, Member 19) is a Protein Coding gene. Diseases associated with DNAJC19 include 3-methylglutaconic aciduria, type v and barth syndrome. Among its related pathways are Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein and Mitochondrial Protein Import. An important paralog of this gene is DNAJC15.
UniProtKB/Swiss-Prot for DNAJC19 Gene
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).