Recombinant Human Mitochondrial import inner membrane translocase subunit TIM14 (DNAJC19)

Uniprot Id

Q96DA6

Target Species

Human

Target Name

DNAJC19

Target Full Name

Mitochondrial import inner membrane translocase subunit TIM14

Target Function

Mitochondrial co-chaperone which forms a complex with prohibitins to regulate cardiolipin remodeling. May be a component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. May act as a co-chaperone that stimulate the ATP-dependent activity.

Target Involvement

3-methylglutaconic aciduria 5 (MGA5)

Target Subcellular Location

Mitochondrion inner membrane; Single-pass membrane protein; Matrix side.

Target Protein Families

TIM14 family

Target Tissue Specificity

Ubiquitously expressed.

Target Research Area

Signal Transduction

Target Synonyms

DnaJ (Hsp40) homolog; subfamily C; member 19; DnaJ homolog subfamily C member 19; DNAJC19; homolog of yeast TIM14; Mitochondrial import inner membrane translocase subunit TIM14; PAM18; TIM 14; TIM14; TIM14_HUMAN; TIMM 14; TIMM14; translocase of the inner mitochondrial membrane 14; Translocase of the inner mitochondrial membrane 14; yeast homolog of

Target Background

The protein encoded by this gene 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). Alternative splicing of this gene results in multiple transcript variants. Related pseudogenes have been identified on chromosomes 1, 2, 6, 10, 14 and 19.