Leucine-rich repeat and sterile alpha motif containing 1 (LRSAM1)

Leucine-rich repeat and sterile alpha motif containing 1 (LRSAM1) is a cytoplasmic E3 ubiquitin-protein ligase highly expressed in spinal cord motoneurons and developing muscle tissue[5][7]. It features leucine-rich repeats, a RING-type zinc finger, and a sterile alpha motif (SAM) domain[5]. LRSAM1 mediates monoubiquitination of TSG101, thereby regulating endocytic and exocytic cargo sorting and participating in protein degradation pathways[7][9]. It is involved in immune defense, clearing intracellular bacteria through the autophagy pathway, and has been implicated in cancer cell growth and neuron health[7][5]. Mutations in LRSAM1 are causative for the inherited axonal neuropathy Charcot-Marie-Tooth disease type 2P[1][7][9]. Its precise role as a therapeutic target is under investigation, and there are currently no directly approved drugs targeting LRSAM1.

Other names

E3 ubiquitin-protein ligase LRSAM1RING finger leucine repeat richCMT2PFLJ31641RIFLETALTSG101-associated ligasehTAL

Targeted by hypothetical modulators of E3 ubiquitin ligase activity (therapeutics may inhibit or enhance ubiquitination-mediated processes); specific approved interacting drugs not established