ST14
Function
Exhibits trypsin-like activity as defined by cleavage of synthetic substrates with Arg or Lys as the P1 site (PubMed:10373424). Involved in the terminal differentiation of keratinocytes through prostasin (PRSS8) activation and filaggrin (FLG) processing (PubMed:18843291). Proteolytically cleaves and therefore activates TMPRSS13 (PubMed:28710277).
Involvement in disease
Ichthyosis, congenital, autosomal recessive 11
ARCI11
A form of autosomal recessive congenital ichthyosis, a disorder of keratinization with abnormal differentiation and desquamation of the epidermis, resulting in abnormal skin scaling over the whole body. The main skin phenotypes are lamellar ichthyosis (LI) and non-bullous congenital ichthyosiform erythroderma (NCIE), although phenotypic overlap within the same patient or among patients from the same family can occur. Lamellar ichthyosis is a condition often associated with an embedment in a collodion-like membrane at birth; skin scales later develop, covering the entire body surface. Non-bullous congenital ichthyosiform erythroderma characterized by fine whitish scaling on an erythrodermal background; larger brownish scales are present on the buttocks, neck and legs.
None
The disease is caused by variants affecting the gene represented in this entry.
Sequence Similarities
Belongs to the peptidase S1 family.
Cellular localization
- Membrane
- Single-pass type II membrane protein
Alternative names
PRSS14, SNC19, TADG15, ST14, Suppressor of tumorigenicity 14 protein, Matriptase, Membrane-type serine protease 1, Prostamin, Serine protease 14, Serine protease TADG-15, Tumor-associated differentially-expressed gene 15 protein, MT-SP1