LRSAM1 (leucine-rich repeat and sterile α motif-containing protein 1), also known as Tsg101-associated ligase, is a 723 amino acid E3 ubiquitin-protein ligase that mediates the monoubiquination of tsg 101, a regulator of vesicular trafficking. This leads to inactivation of the sorting of endocytic and exocytic cargos, such as EGF receptors and HIV-1 viral proteins. Mutations within the LRSAM1 gene have been identified in patients with Charcot-Marie-Tooth disease. There are three isoforms of LRSAM1 that are produced as a result of alternative splicing events.
