Automated description from the Alliance of Genome Resources (Release 7.1.0)
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 protein ubiquitination; and proteolysis involved in protein catabolic process. Acts upstream of or within protein polyubiquitination. Located in Z disc. Is expressed in tooth. 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).