Background: The majority of mitochondrial-directed proteins are encoded by the nuclear genome and are transported to the mitochondria via regulated processes involving the mitochondrial Tom and Tim proteins. The mitochondrial Tim protein family is comprised of a large group of evolutionarily conserved proteins that are found in most eukaryotes and are thought to play a role in health and development. Tim14, also known as DNAJC19 (DnaJ (Hsp40) homolog, subfamily C, member 19), is a 116 amino acid single-pass membrane protein that localizes to the inner membrane of the mitochondrion and contains one J domain. Expressed ubiquitously, Tim14 functions as a component of the mitochondrial TIM23 complex, which is responsible for the ATP-dependent translocation of select proteins from the inner mitochondrial membrane to the mitochondrial matrix. Defects in the gene encoding Tim14 are the cause of 3-methylglutaconic aciduria type 5 (MGA5), an autosomal recessive disorder characterized by testicular dysgenesis, dilated cardiomyopathy, growth failure and cerebellar ataxia causing significant motor delays.
Description: Rabbit polyclonal to DNAJC13
Immunogen: KLH conjugated synthetic peptide derived from DNAJC19
Specificity: ·Reacts with Human, Mouse and Rat.
·Isotype: IgG
Application: ·Western blotting: 1/100-500. Predicted Mol wt: 12 kDa;
·Immunohistochemistry (Paraffin/frozen tissue section): 1/50-200;
·Immunocytochemistry/Immunofluorescence: 1/100;
·Immunoprecipitation: 1/50;
·ELISA: 1/500;
·Optimal working dilutions must be determined by the end user.
