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Genetics Home Reference: your guide to understanding genetic conditions     A service of the U.S. National Library of Medicine®


Reviewed November 2007

What is the official name of the DNAJC19 gene?

The official name of this gene is “DnaJ (Hsp40) homolog, subfamily C, member 19.”

DNAJC19 is the gene's official symbol. The DNAJC19 gene is also known by other names, listed below.

What is the normal function of the DNAJC19 gene?

The DNAJC19 gene provides instructions for producing a protein that is active in the energy-producing centers in cells (mitochondria). Researchers believe that this protein helps transport other proteins into and out of these cellular structures. It is also thought that the DNAJC19 protein assists in the proper assembly and disassembly of some proteins.

Does the DNAJC19 gene share characteristics with other genes?

The DNAJC19 gene belongs to a family of genes called DNAJ (heat shock proteins, DNAJ (HSP40)).

A gene family is a group of genes that share important characteristics. Classifying individual genes into families helps researchers describe how genes are related to each other. For more information, see What are gene families? ( in the Handbook.

How are changes in the DNAJC19 gene related to health conditions?

3-methylglutaconic aciduria - caused by mutations in the DNAJC19 gene

At least one mutation in the DNAJC19 gene has been shown to cause 3-methylglutaconic aciduria type V, also called dilated cardiomyopathy with ataxia (DCMA). The known mutation changes one DNA building block (nucleotide) in the gene. Specifically, the mutation substitutes the nucleotide guanine for the nucleotide cytosine near the end of an area of the gene called exon 3 (written as IVS3-1G>C). This genetic change causes a disruption in the way the gene's instructions are used to make the DNAJC19 protein. 3-methylglutaconic aciduria type V is seen only in the genetically isolated Hutterite population of Canada and the northern United States. Researchers have not determined how the IVS3-1G>C mutation leads to the signs and symptoms of this disorder.

Where is the DNAJC19 gene located?

Cytogenetic Location: 3q26.33

Molecular Location on chromosome 3: base pairs 180,983,708 to 180,989,773

The DNAJC19 gene is located on the long (q) arm of chromosome 3 at position 26.33.

The DNAJC19 gene is located on the long (q) arm of chromosome 3 at position 26.33.

More precisely, the DNAJC19 gene is located from base pair 180,983,708 to base pair 180,989,773 on chromosome 3.

See How do geneticists indicate the location of a gene? ( in the Handbook.

Where can I find additional information about DNAJC19?

You and your healthcare professional may find the following resources about DNAJC19 helpful.

You may also be interested in these resources, which are designed for genetics professionals and researchers.

What other names do people use for the DNAJC19 gene or gene products?

  • homolog of yeast TIM14
  • mitochondrial import inner membrane translocase subunit TIM 14
  • TIM14
  • TIMM14
  • translocase of the inner mitochondrial membrane 14

See How are genetic conditions and genes named? ( in the Handbook.

What glossary definitions help with understanding DNAJC19?

aciduria ; ataxia ; cardiomyopathy ; cytosine ; dilated ; DNA ; exon ; gene ; guanine ; mitochondria ; mutation ; nucleotide ; population ; protein ; subunit

You may find definitions for these and many other terms in the Genetics Home Reference Glossary (


  • Davey KM, Parboosingh JS, McLeod DR, Chan A, Casey R, Ferreira P, Snyder FF, Bridge PJ, Bernier FP. Mutation of DNAJC19, a human homologue of yeast inner mitochondrial membrane co-chaperones, causes DCMA syndrome, a novel autosomal recessive Barth syndrome-like condition. J Med Genet. 2006 May;43(5):385-93. Epub 2005 Jul 31. (
  • MacKenzie JA, Payne RM. Mitochondrial protein import and human health and disease. Biochim Biophys Acta. 2007 May;1772(5):509-23. Epub 2006 Dec 9. Review. (
  • NCBI Gene (
  • Sparkes R, Patton D, Bernier F. Cardiac features of a novel autosomal recessive dilated cardiomyopathic syndrome due to defective importation of mitochondrial protein. Cardiol Young. 2007 Apr;17(2):215-7. Epub 2007 Jan 23. (


The resources on this site should not be used as a substitute for professional medical care or advice. Users seeking information about a personal genetic disease, syndrome, or condition should consult with a qualified healthcare professional. See How can I find a genetics professional in my area? ( in the Handbook.

Reviewed: November 2007
Published: April 21, 2014