DNAJC19 : DnaJ heat shock protein family (Hsp40) member C19
Description
The DNAJC19 (DnaJ heat shock protein family (Hsp40) member C19) is a protein-coding gene located on chromosome 3.
The DNAJC19 gene provides instructions for making a protein found in mitochondria, the energy-producing centers of cells. Although its exact role is unclear, scientists believe it helps transport other proteins into and out of mitochondria and may be involved in assembling and disassembling specific proteins.
DNAJC19 is a mitochondrial co-chaperone that may play a role in regulating cardiolipin remodeling by forming a complex with prohibitins. It may also be a component of the PAM complex, which is involved in the translocation of proteins from the mitochondrial inner membrane into the matrix in an ATP-dependent manner. As a co-chaperone, DNAJC19 may stimulate ATP-dependent activity.
DNAJC19 is also known as PAM18, TIM14, TIMM14.
Associated Diseases
- 3-methylglutaconic aciduria, type V
- Dilated cardiomyopathy with ataxia
- Dilated cardiomyopathy with ataxia syndrome
