ELANE (also known as ELA2 or NE), a human neutrophil elas...

ELANE (also known as ELA2 or NE), a human neutrophil elastase, is a serine protease that interacts with NOTCH2NLA (also known as N2N or NOTCH2NL) and cleaves it within EGF repeats (Duan et al. 2004). ELANE-mediated proteolysis of NOTCH2NLA is slowed down by the C-terminus of NOTCH2NLA (Duan et al. 2004). Some of the ELANE mutations that cause cyclic and severe congenital neutropenia disrupt the interaction between NOTCH2NLA and ELANE and impair ELANE-mediated NOTCH2NLA proteolysis (Duan et al. 2004). NOTCH2 may also be cleaved by ELANE (Duan et al. 2004). Wild type ELANE inhibits NOTCH2 transcriptional activity in a dose-dependent manner (Duan et al. 2004).