USP13A gene on chromosome 3q26.2-q26.3 that encodes a deubiquitinating enzyme that mediates deubiquitination of target proteins–e.g., BECN1, a key regulator of autophagy, stabilising PIK3C3/VPS34-containing complexes which in turn regulate USP13 stability and possibly p53/TP53 by regulating USP10 stability. USP13 is recruited by nuclear UFD1 and deubiquitinates SKP2, thereby regulating endoplasmic reticulum-associated degradation—ERAD. USP13 also stabilises SIAH2 independently of deubiquitinase activity, by binding to ubiquitinated SIAH2 and impairing its autoubiquitination.
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