DNAJC19, Polyclonal Antibody

Mitochondrial import inner membrane translocase subunit TIM14; DnaJ homolog subfamily C member 19; DNAJC19; TIM14; TIMM14

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

Polyclonal

Rabbit Ig

Rabbit

This DNAJC19 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 55-84 amino acids from the Central region of human DNAJC19.

Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

Vial Concentration: 2 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-DNAJC19 antibody 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.

DNAJC19 is a 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. The protein may act as a co-chaperone that stimulate the ATP-dependent activity.

Neuroscience; Signal Transduction

Western Blot (WB), ELISA (EIA)

WB~~1:1000

Western blot analysis of DNAJC19 Antibody (Center) in mouse kidney tissue lysates (35ug/lane). DNAJC19 (arrow) was detected using the purified Pab.

12499

DnaJ (Hsp40) homolog, subfamily C, member 19

mitochondrial import inner membrane translocase subunit 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: Membrane protein, integral; Mitochondrial; Chaperone

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

Sparkes,R., Cardiol Young 17 (2), 215-217 (2007)
Davey,K.M., J. Med. Genet. 43 (5), 385-393 (2006)
Mokranjac,D., EMBO J. 22 (19), 4945-4956 (2003)

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