biostudies-literatureKarlsson ABNINDS NIH HHSNIGMS NIH HHS1355-65https://www.ebi.ac.uk/biostudies/studies/S-EPMC3982999biostudies-literatureUnknown25(8)Troyer syndrome is an autosomal recessive hereditary spastic paraplegia (HSP) caused by frameshift mutations in the SPG20 gene that results in a lack of expression of the truncated protein. Spartin is a multifunctional protein, yet only two conserved domains--a microtubule-interacting and trafficking domain and a plant-related senescence domain involved in cytokinesis and mitochondrial physiology, respectively--have been defined. We have shown that overexpressed spartin binds to the Ile44 hydrophobic pocket of ubiquitin, suggesting spartin might contain a ubiquitin-binding domain. In the present study, we demonstrate that spartin contributes to the formation of dendritic aggresome-like induced structures (DALIS) through a unique ubiquitin-binding region (UBR). Using short hairpin RNA, we kMolecular biology of the cellThe role of spartin and its novel ubiquitin binding region in DALIS occurrence.PMC3982999R01 GM0850061R01NS073967-01A1R01 NS073967Hooper CShekhtman AKarlsson ABDimitrova VWashington JBakowska JCfalseThe role of spartin and its novel ubiquitin binding region in DALIS occurrence.Troyer syndrome is an autosomal recessive hereditary spastic paraplegia (HSP) caused by frameshift mutations in the SPG20 gene that results in a lack of expression of the truncated protein. Spartin is a multifunctional protein, yet only two conserved domains--a microtubule-interacting and trafficking domain and a plant-related senescence domain involved in cytokinesis and mitochondrial physiology, respectively--have been defined. We have shown that overexpressed spartin binds to the Ile44 hydrophobic pocket of ubiquitin, suggesting spartin might contain a ubiquitin-binding domain. In the present study, we demonstrate that spartin contributes to the formation of dendritic aggresome-like induced structures (DALIS) through a unique ubiquitin-binding region (UBR). Using short hairpin RNA, we k2014-01-01T00:00:00Z2014 Apr2025-04-18T17:36:13.064Z2019-03-27T01:24:46ZS-EPMC39829992452328610.1091/mbc.E13-11-0705