Description
DNAJC19 is a single-pass membrane protein that contains a J domain and is localized to the inner membrane of the mitochondrion. Expressed ubiquitously, DNAJC19 functions as a component of the mitochondrial DNAJC19 complex, which is responsible for the ATP-dependent translocation of select proteins from the inner mitochondrial membrane to the mitochondrial matrix. Defects in DNAJC19 are the cause of 3-methylglutaconic aciduria type 5 (MGA5), also known as dilated cardiomyopathy with ataxia (DCMA). Recombinant human DNAJC19 protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques