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LRSAM1 E3 ubiquitin-protein ligase that mediates monoubiquitination of TSG101 at multiple sites, leading to inactivate the ability of TSG101 to sort endocytic (EGF receptors) and exocytic (HIV-1 viral proteins) cargos. Defects in LRSAM1 are a cause of Charcot-Marie-Tooth disease type 2P (CMT2P). CMT2P is an axonal form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Neuropathies of the CMT2 group are characterized by signs of axonal degeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. Nerve conduction velocities are normal or slightly reduced. 3 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: EC 6.3.2.-; EC; Ligase; Ubiquitin conjugating system; Ubiquitin ligase
Chromosomal Location of Human Ortholog: 9q33.3-q34.11
Cellular Component: cytoplasm; cytosol; membrane; plasma membrane
Molecular Function: metal ion binding; protein binding; ubiquitin-protein transferase activity; volume-sensitive anion channel activity
Biological Process: autophagy; negative regulation of endocytosis; non-lytic virus budding; protein autoubiquitination; protein catabolic process; protein polyubiquitination; signal transduction
Disease: Charcot-marie-tooth Disease, Axonal, Type 2p
Reference #:  Q6UWE0 (UniProtKB)
Alt. Names/Synonyms: E3 ubiquitin-protein ligase LRSAM1; FLJ31641; hTAL; leucine rich repeat and sterile alpha motif containing 1; Leucine-rich repeat and sterile alpha motif-containing protein 1; LRSAM1; LRSM1; RIFLE; TAL; Tsg101-associated ligase
Gene Symbols: LRSAM1
Molecular weight: 83,594 Da
Basal Isoelectric point: 5.7  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
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Protein Structure Not Found.

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