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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 domain. It can act as a transcriptional corepressor of p45/NFE2 and may participate in the regulation of immune responses by modifying Notch-mediated signaling. It is highly similar to the mouse Itch protein, which has been implicated in the regulation and differentiation of erythroid and lymphoid cells.
6720481N21Rik; 8030492O04Rik; A130065M08; ADMFD; AIF4; AIP4; atrophin-1 interacting protein 4; Atrophin-1-interacting protein 4; C230047C07Rik; dJ468O1.1; E3 ubiquitin-protein ligase Itchy; E3 ubiquitin-protein ligase Itchy homolog; HECT-type E3 ubiquitin transferase Itchy homolog; ITCH; itch E3 ubiquitin ligase; itchy E3 ubiquitin protein ligase; itchy E3 ubiquitin protein ligase homolog; itchy, E3 ubiquitin protein ligase; NAPP1; NFE2-associated polypeptide 1
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