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DNAJC19 Polyclonal Antibody
Purified Rabbit Polyclonal Antibody (Pab)
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- BACKGROUND
Application 
| IHC-P, IHC-F, IF, ICC |
|---|---|
| Primary Accession | Q96DA6 |
| Reactivity | Rat, Pig, Dog, Bovine |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 12 KDa |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human DNAJC19 |
| Epitope Specificity | 21-116/116 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SUBCELLULAR LOCATION | Mitochondrion inner membrane. |
| SIMILARITY | Belongs to the TIM14 family. Contains 1 J domain. |
| DISEASE | Defects in DNAJC19 are the cause of 3-methylglutaconic aciduria type 5 (MGA5) [MIM:610198]; 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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] |
| Gene ID | 131118 |
|---|---|
| Other Names | Mitochondrial import inner membrane translocase subunit TIM14, DnaJ homolog subfamily C member 19, DNAJC19, TIM14, TIMM14 |
| Target/Specificity | Ubiquitously expressed. |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,ICC=1:100-500,IF=1:100-500 |
| Storage | Store at -20 ℃ for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 ℃. |
| Name | DNAJC19 |
|---|---|
| Synonyms | TIM14, TIMM14 |
| Function | Mitochondrial co-chaperone which forms a complex with prohibitins to regulate cardiolipin remodeling (By similarity). 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 (By similarity). |
| Cellular Location | Mitochondrion inner membrane; Single-pass membrane protein; Matrix side {ECO:0000250|UniProtKB:Q9CQV7} |
| Tissue Location | Ubiquitously expressed. |
Research Areas
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Application Protocols
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$ 385.00
Cat# AP55552
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