DnaJ/HSP40 Homolog Subfamily C, Member 19 (DNAJC19)

DNAJC19 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). Several transcript variants, some protein-coding and some not, have been found for this gene.The deduced 116-amino acid protein contains an N-terminal transmembrane domain, followed by 3 alpha-helical segments characteristic of the J domain of E. coli DnaJ . In yeast, Tim14 is located at the inner mitochondrial membrane and the J domain faces the matrix space.