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. 2015 Aug 20:650–660. doi: 10.1016/B978-0-12-394447-4.10076-8

Table 1.

Membrane-anchored serine proteases – physiological functions learnt through protease deficiency

Protease Function Implicated substrate References
GPI-anchored
 Prostasin Maintenance of epidermal barrier function Unknown (Frateschi et al., 2012, Leyvraz et al., 2005, Peters et al., 2014)
Regulation of ENaC-mediated fluid clearance in lung and colon epithelium ENaC-γ subunit (Frateschi et al., 2012, Malsure et al., 2014, Planes et al., 2010)
Regulation of hepatic insulin sensitivity by cleavage and inactivation of TLR-4 Toll-like receptor-4 (TLR-4) (Uchimura et al., 2014)
 Testisin Involved in sperm cell maturation and fertilizing ability Unknown (Aimes et al., 2003, Inoue et al., 1998, Netzel-Arnett et al., 2009, Yamashita et al., 2008)


Type II
HAT/DESC subfamily
 HAT Unknown. Not required for development, long-term health or survival in mice Unknown (Bertram et al., 2012, Iwakiri et al., 2004, Takahashi et al., 2001, Yamaoka et al., 1998)
 HATL-1 Unknown. Not required for development, long-term health or survival in mice Unknown (Faller et al., 2014, Kam et al., 2009, Sales et al., 2011)
Hepsin/TMPRSS subfamily
 Hepsin Important for hearing in mice, where it has a role in cochlear development in inner ear Unknown (Faller et al., 2014, Guipponi et al., 2007, Kurachi et al., 1994, Tsuji et al., 1991)
Maintenance of liver structural homeostasis in mice Pro-HGF (Hsu et al., 2012)
 TMPRSS2 Unknown. Not required for development, long-term health, fertility or survival in mice Unknown (Faller et al., 2014, Jacquinet et al., 2001, Kim et al., 2006)
 TMPRSS3 Mediates normal hearing in humans. Shown to be critical for cochlear hair cell survival in murine model of deficiency Unknown (Fasquelle et al., 2011, Guipponi et al., 2008, Lee et al., 2013, Molina et al., 2013)
 TMPRSS5 Mediates normal hearing in humans Unknown (Guipponi et al., 2008, Yamaguchi et al., 2002)
 Enteropeptidase Critical for digestion of dietary proteins. Initiates a proteolytic cascade that results in the activation of several intestinal proteases Trypsinogen (Haworth et al., 1971, Zheng et al., 2009)
Matriptase subfamily
 Matriptase Global role in maintenance of epidermal and epithelial barrier function and homeostasis. Unknown (List et al., 2002, List et al., 2009, Yin et al., 2014)
 Matriptase-2 Essential regulator of iron homeostasis that prevents iron deficiency anemia Hemojuvelin (Du et al., 2008, Folgueras et al., 2008, Truksa et al., 2009)
Corin subfamily
 Corin Regulates systemic salt and water balance to prevent hypertension and cardiac hypertrophy Pro-ANP, pro-BNP (Chan et al., 2005, Rame et al., 2009, Wang et al., 2008)