E3 ubiquitin-protein ligase which is a component of the N-end rule pathway. Recognizes and binds to proteins bearing specific N-terminal residues that are destabilizing according to the N-end rule, leading to their ubiquitination and subsequent degradation. May be involved in pancreatic homeostasis. Binds leucine and is a negative regulator of the leucine-mTOR signaling pathway, thereby controlling cell growth.Kwak K.S., Cancer Res. 64:8193-8198(2004).Dgany O., Am. J. Hum. Genet. 71:1467-1474(2002).Ota T., Nat. Genet. 36:40-45(2004).