Atrophin-1 contains a polyglutamine repeat, expansion of which is responsible for dentatorubral and pallidoluysian atrophy. The protein encoded by this gene interacts with atrophin-1. This encoded protein is a closely related member of the NEDD4-like protein family. This family of proteins are E3 ubiquitin-ligase molecules and regulate key trafficking decisions, including targeting of proteins to proteosomes or lysosomes. This encoded protein contains four tandem WW domains and a HECT (homologous to the E6-associated protein carboxyl terminus) domain.
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