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SerpinB6 Protein, Antibody, ELISA Kit, cDNA Clone

SerpinB6 Related Areas

SerpinB6 Related Pathways

SerpinB6 Related Product

    SerpinB6 Summary & Protein Information

    SerpinB6 Background

    Gene Summary: The protein encoded by SERPINB6 gene is a member of the serpin (serine proteinase inhibitor) superfamily, and ovalbumin(ov)-serpin subfamily. It was originally discovered as a placental thrombin inhibitor. The mouse homolog was found to be expressed in the hair cells of the inner ear. Mutations in SERPINB6 gene are associated with nonsyndromic progressive hearing loss, suggesting that this serpin plays an important role in the inner ear in the protection against leakage of lysosomal content during stress, and that loss of this protection results in cell death and sensorineural hearing loss. Alternatively spliced transcript variants have been found for SERPINB6 gene. [provided by RefSeq, Sep 2010]
    General information above from NCBI
    Subunit structure: Forms a complex with the monomeric form of beta-tryptase.
    Subcellular location: Cytoplasm.
    Tissue specificity: Expressed in keratinocytes (at protein level). Highest levels in skeletal muscle. Also found in placenta, cardiac muscle, lung, liver, kidney and pancreas. Expressed in the inner ear hair cells. Expressed abundantly by normal mast cells in different tissues and by mast cells in mastocytoma lesions.
    Involvement in disease: Deafness, autosomal recessive, 91 (DFNB91) [MIM:613453]: A form of non-syndromic deafness characterized by progressive and age-dependent sensorineural hearing loss. Vestibular function is normal. Note=The disease is caused by mutations affecting the gene represented in this entry.
    Sequence similarity: Belongs to the serpin family. Ov-serpin subfamily.
    General information above from UniProt

    SerpinB6, also known as Cytoplasmic antiproteinase, Peptidase inhibitor 6, Placental thrombin inhibitor, SERPINB6 and PI-6, is a cytoplasm protein which belongs to the serpin family and Ov-serpin subfamily. SerpinB6 / PI-6 is an inhibitor of cathepsin G, kallikrein-8 and thrombin. It may play an important role in the inner ear in the protection against leakage of lysosomal content during stress and loss of this protection results in cell death and sensorineural hearing loss. SerpinB6 / PI-6 is expressed in keratinocytes (at protein level). It is also found in placenta, cardiac muscle, lung, liver, kidney and pancreas. SerpinB6 / PI-6 is expressed in the inner ear hair cells. It expressed abundantly by normal mast cells in different tissues and by mast cells in mastocytoma lesions. SerpinB6 / PI-6 may be involved in the regulation of serine proteinases present in the brain or extravasated from the blood. Defects in SerpinB6 are the cause of deafness autosomal recessive type 91 which is a form of non-syndromic deafness characterized by progressive and age-dependent sensorineural hearing loss. Vestibular function is normal.

    SerpinB6 Alternative Name

    MGC111370,MSTP057,PI6,PTI,RP1-90J20.6,SerpinB6,SPI3,CAP,DFNB91,DKFZp686I04222, [human]
    D330015H01Rik,RP23-262J21.1,Serpinb6,4930482L21Rik,AI876477,ovalbumin,Serpinb6a,Spi3, [mouse]

    SerpinB6 Related Studies

  • Morgenstern KA. et al.,1994, Biochemistry. 33: 3432-41.
  • Strik MC. et al., 2004, Blood. 103: 2710-7.
  • Scott FL. et al., 2007, J Biochem. 142: 435-42.
  • Burkard TR. et al., 2011, BMC Syst Biol. 5:17.
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