DNAJC19 Human; DnaJ (Hsp40) Homolog, Subfamily C, Member 19 Human Recombinant; Mitochondrial import inner membrane translocase subunit TIM14; DnaJ homolog subfamily C member 19; DNAJC19; TIM14; TIMM14; Pam18

For Research Use Only. Not for use in diagnostic procedures.

E Coli

Greater than 90.0% as determined by SDS-PAGE.

The DNAJC19 solution (1 mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 10% glycerol, 2mM DTT and 0.1M NaCl.
Sterile Filtered colorless solution.

Store at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.

Small volumes of DNAJC19 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Description: DNAJC19 Human Recombinant fused with a 37 amino acid His tag at N-terminus produced in E Coli is a single, non-glycosylated, polypeptide chain containing 135 amino acids (19-116 a.a.) and having a molecular mass of 15.1kDa. The DNAJC19 is purified by proprietary chromatographic techniques.

Introduction: DNAJC19 is part of a complex involved in the ATP-dependent transport of transit peptide-containing proteins from the inner cell membrane to the mitochondrial matrix. DNAJC19 is a single-pass membrane protein which contains a J domain and is localized to the inner membrane of the mitochondrion. Expressed ubiquitously, DNAJC19 acts as a component of the mitochondrial DNAJC19 complex that is responsible for the ATP-dependent translocation of select proteins from the inner mitochondrial membrane to the mitochondrial matrix. Defects in the DNAJC19 gene are the cause of 3-methylglutaconic aciduria type 5 (MGA5), otherwise known as dilated cardiomyopathy with ataxia (DCMA).

HEAT SHOCK PROTEINS; Recombinant Proteins; Heat Shock Protein

10,079 Da

DnaJ (Hsp40) homolog, subfamily C, member 19

mitochondrial import inner membrane translocase subunit TIM14; homolog of yeast TIM14

Mitochondrial import inner membrane translocase subunit TIM14

TIM14; TIMM14??[Similar Products]

TIM14_HUMAN

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. [provided by RefSeq, Jan 2012]

DNAJC19: Probable 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. Defects in DNAJC19 are the cause of 3-methylglutaconic aciduria type 5 (MGA5); also known as dilated cardiomyopathy with ataxia (DCMA). MGA5 is an autosomal recessive disorder characterized by early-onset dilated cardiomyopathy, growth failure, cerebellar ataxia causing significant motor delays, testicular dysgenesis, growth failure, and significant increases in urine organic acids, particularly 3-methylglutaconic acid and 3-methylglutaric acid. Belongs to the TIM14 family.

Protein type: Chaperone; Mitochondrial; Membrane protein, integral

Chromosomal Location of Human Ortholog: 3q26.33

Cellular Component: protein complex; mitochondrion; mitochondrial inner membrane; integral to membrane

Molecular Function: protein binding

Biological Process: genitalia development; cellular protein metabolic process; visual perception; protein folding; protein targeting to mitochondrion

Disease: 3-methylglutaconic Aciduria, Type V

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