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PJVK Antibody (Center) Blocking Peptide
Synthetic peptide
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
| Primary Accession | Q0ZLH3 |
|---|---|
| Clone Names | 110729178 |
| Gene ID | 494513 |
|---|---|
| Other Names | Pejvakin, Autosomal recessive deafness type 59 protein, DFNB59, PJVK |
| Format | Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed. |
| Storage | Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C. |
| Precautions | This product is for research use only. Not for use in diagnostic or therapeutic procedures. |
| Name | PJVK {ECO:0000303|PubMed:16804542, ECO:0000312|HGNC:HGNC:29502} |
|---|---|
| Function | Peroxisome-associated protein required to protect auditory hair cells against noise-induced damage. Acts by regulating noise- induced peroxisome proliferation in auditory hair cells and neurons, and promoting autophagic degradation of damaged peroxisomes (pexophagy). Noise overexposure increases reactive oxygen species (ROS) levels, causing oxidative damage to auditory hair cells and resulting in hearing loss. PJVK acts as a ROS sensor that recruits the autophagy machinery to trigger pexophagy of peroxisomes damaged by oxidative stress. In addition to pexophagy, also required to promote peroxisome proliferation in response to sound overstimulation. |
| Cellular Location | Peroxisome membrane {ECO:0000250|UniProtKB:Q0ZLH2}. Cell projection, cilium {ECO:0000250|UniProtKB:Q0ZLH2}. Note=Associates with the peroxisomal membrane; it is unclear whether it is embedded or just associated with the peroxisomal membrane. Localizes to ciliary rootlet {ECO:0000250|UniProtKB:Q0ZLH2} |
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Provided below are standard protocols that you may find useful for product applications.
Background
The protein encoded by this gene is a member of thegasdermin family, a family which is found only in vertebrates. Theencoded protein is required for the proper function of auditorypathway neurons. Defects in this gene are a cause of non-syndromicsensorineural deafness autosomal recessive type 59 (DFNB59).
References
Mahdieh, N., et al. J. Hum. Genet. 55(10):639-648(2010)Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)Hashemzadeh Chaleshtori, M., et al. Clin. Genet. 72(3):261-263(2007)Collin, R.W., et al. Hum. Mutat. 28(7):718-723(2007)
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