Rabbit anti-Human, Mouse DNAJC19 Polyclonal Antibody | anti-DNAJC19 antibody

DNAJC19, ID (Mitochondrial Import Inner Membrane Translocase Subunit TIM14, DnaJ Homolog Subfamily C Member 19, TIMM14, TIM14, DNAJC19) (MaxLight 750)

Cat. Num. AAA6472722

• Gene Names: DNAJC19; PAM18; TIM14; TIMM14
• Reactivity: Human, Mouse
• Applications: Western Blot
• Purity: Purified by Ammonium Sulfate Precipitation.
• Synonyms: DNAJC19; Polyclonal Antibody; ID (Mitochondrial Import Inner Membrane Translocase Subunit TIM14; DnaJ Homolog Subfamily C Member 19; TIMM14; TIM14; DNAJC19) (MaxLight 750); anti-DNAJC19 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human DNAJC19. Species Crossreactivity: mouse.
• Purity/Purification: Purified by Ammonium Sulfate Precipitation.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight750.

• Applicable Applications for anti-DNAJC19 antibody: Western Blot (WB), FLISA
• Application Notes: WB: 1:50-1:100; FLISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the center region of human DNAJC19 (KLH).
• Conjugate: MaxLight750
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight750 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

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

Product Categories/Family for anti-DNAJC19 antibody

Antibodies; Membrane Proteins

References

1. Sparkes, R., Cardiol Young 17(2), 215-217 (2007). 2. Davey, K.M., J. Med. Genet. 43(5), 385-393 (2006). 3. Mokranjac, D., EMBO J. 22(19), 4945-4956 (2003).

NCBI and Uniprot Product Information

• NCBI GI #: 21687102
• NCBI GeneID: 131118
• NCBI Accession #: NP_660304
• NCBI GenBank Nucleotide #: NM_145261.3
• UniProt Accession #: Q96DA6; B2R4B1; C9JBV1
• Molecular Weight: 15.1 kDa (135aa), confirmed by MALDI-TOF
• NCBI Official Full Name: mitochondrial import inner membrane translocase subunit TIM14 isoform 1
• NCBI Official Synonym Full Names: DnaJ heat shock protein family (Hsp40) member C19
• NCBI Official Symbol: DNAJC19
• NCBI Official Synonym Symbols: PAM18; TIM14; TIMM14
• NCBI Protein Information: mitochondrial import inner membrane translocase subunit TIM14
• UniProt Protein Name: Mitochondrial import inner membrane translocase subunit TIM14
• UniProt Gene Name: DNAJC19
• UniProt Synonym Gene Names: TIM14; TIMM14
• UniProt Entry Name: TIM14_HUMAN

NCBI Description

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]

Uniprot Description

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