Stub1 - STIP1 homology and U-Box containing protein 1

Enables protein homodimerization activity; ubiquitin protein ligase binding activity; and ubiquitin-ubiquitin ligase activity. Involved in several processes, including cellular response to topologically incorrect protein; positive regulation of ubiquitin-protein transferase activity; and proteolysis involved in cellular protein catabolic process. Acts upstream of or within protein polyubiquitination. Located in Z disc. Used to study autosomal recessive spinocerebellar ataxia 16. Human ortholog(s) of this gene implicated in autosomal recessive spinocerebellar ataxia 16 and cerebellar ataxia type 48. Orthologous to human STUB1 (STIP1 homology and U-box containing protein 1). [provided by Alliance of Genome Resources, Apr 2022]