DNAJC19 - Revision history

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'''DNAJC19''' is a protein that in humans is encoded by the DNAJC19 gene. This gene is located on the [[chromosome 3]] and is known to be involved in the [[mitochondrial]] protein import and [[cardiomyopathy]].

== Function ==

The DNAJC19 protein is a member of the [[DnaJ]] heat shock protein family. These proteins are characterized by a highly conserved [[amino acid]] sequence, which includes a J-domain. The J-domain is responsible for the interaction with [[Hsp70]] proteins, which are crucial for protein folding, assembly, translocation, and degradation.

DNAJC19 is localized in the [[mitochondria]], where it is involved in the import of proteins into the inner mitochondrial membrane. It is also associated with the [[TIM23]] complex, which is responsible for the translocation of proteins across the mitochondrial inner membrane.

== Clinical significance ==

Mutations in the DNAJC19 gene have been associated with [[Dilated cardiomyopathy]] with ataxia (DCMA), a rare disorder characterized by early onset dilated cardiomyopathy, ataxia, and elevated 3-methylglutaconic acid levels.

== See also ==

* [[DnaJ]]
* [[Hsp70]]
* [[Mitochondria]]
* [[TIM23 complex]]
* [[Dilated cardiomyopathy]]
* [[Ataxia]]

== References ==

<references />

== External links ==

* [https://www.ncbi.nlm.nih.gov/gene/131118 NCBI Gene]
* [https://www.uniprot.org/uniprot/Q96DA6 UniProt]

[[Category:Human proteins]]
[[Category:Genes on human chromosome 3]]
[[Category:Mitochondrial proteins]]
[[Category:Cardiomyopathy]]
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