Matriptase (ST14) Active Protein

The protein encoded by this gene is an epithelial-derived, integral membrane serine protease. This protease forms a complex with the Kunitz-type serine protease inhibitor, HAI-1, and is found to be activated by sphingosine 1-phosphate. This protease has been shown to cleave and activate hepatocyte growth factor/scattering factor, and urokinase plasminogen activator, which suggest the function of this protease as an epithelial membrane activator for other proteases and latent growth factors. The expression of this protease has been associated with breast, colon, prostate, and ovarian tumors, which implicates its role in cancer invasion, and metastasis. [provided by RefSeq, Jul 2008]

ST14: Degrades extracellular matrix. Proposed to play a role in breast cancer invasion and metastasis. Exhibits trypsin-like activity as defined by cleavage of synthetic substrates with Arg or Lys as the P1 site. Defects in ST14 are a cause of ichthyosis autosomal recessive with hypotrichosis (ARIH). ARIH is a skin disorder characterized by congenital ichthyosis associated with the presence of less than the normal amount of hair. Belongs to the peptidase S1 family.

Protein type: EC 3.4.21.109; Membrane protein, integral; Protease

Chromosomal Location of Human Ortholog: 11q24-q25

Cellular Component: extrinsic to plasma membrane; extracellular space; integral to plasma membrane; basolateral plasma membrane; plasma membrane

Molecular Function: serine-type peptidase activity; serine-type endopeptidase activity

Biological Process: keratinocyte differentiation; proteolysis

Disease: Ichthyosis, Congenital, Autosomal Recessive 11