serpin B6; cytoplasmic antiproteinase; CAP; peptidase inhibitor 6; PI-6; placental thrombin inhibitor (SERPINB6, PI6, PTI)

Function: - may be involved in the regulation of serine proteinases - present in the brain or extravasated from the blood - inhibitor of cathepsin G, kallikrein-8 & thrombin - may play an important role in the inner ear in protection against leakage of lysosomal content during stress - loss of this protection results in cell death & sensorineural hearing loss - forms a complex with the monomeric form of beta-tryptase Structure: - belongs to the serpin family, Ov-serpin subfamily Compartment: cytoplasm Expression: - expressed in keratinocytes (at protein level) - highest levels in skeletal muscle - also found in placenta, cardiac muscle, lung, liver, kidney & pancreas - expressed in the inner ear hair cells - expressed abundantly by normal mast cells in different tissues & by mast cells in mastocytoma lesions Pathology: - defects in SERPINB6 are the cause of autosomal recessive deafness type 91

General

secreted protein serine protease inhibitor; serpin

Properties

SIZE: entity length = 376 aa MW = 43 kD COMPARTMENT: cytoplasm MOTIF: serpin motif FOR-BINDING-OF: serine protease peptide motif {341-342} INHIBITS: serine protease

Database Correlations

OMIM correlations UniProt P35237 Pfam PF00079 Entrez Gene 5269 Kegg hsa:5269

References

UniProt :accession P35237