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), as initially detected in a yeast two hybrid screen and confirmed in vitro, using GST-tagged NOTCH2NLA and radioactively labeled ELANE (Duan et al. 2004). The experimental evidence suggests that the C-terminus of ELANE is involved in NOTCH2NLA binding (Duan et al. 2004). The C-terminus of NOTCH2NLA is required but not sufficient for the interaction with ELANE (Duan et al. 2004).
Disease variants involving the C-terminus of ELANE, which are the underlying cause of human hereditary neutropenia, diminish the interaction between ELANE and NOTCH2NLA (Duan et al. 2004).
Duan, Z, Li, FQ, Wechsler, J, Meade-White, K, Williams, K, Benson, KF, Horwitz, M
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