OTUD3


Description

The OTUD3 (OTU deubiquitinase 3) is a protein-coding gene located on chromosome 1.

OTUD3 is a deubiquitinating enzyme that removes ubiquitin chains from proteins. It specifically targets 'Lys-6' and 'Lys-11'-linked polyubiquitin chains, as well as heterotypic (mixed and branched) and homotypic chains. OTUD3 plays a significant role in regulating energy metabolism, being activated by glucose and fatty acids, which trigger its nuclear translocation through CBP-dependent acetylation. Inside the nucleus, OTUD3 stabilizes the nuclear receptor PPARD by removing ubiquitin tags, thus influencing the expression of genes involved in glucose and lipid metabolism and oxidative phosphorylation. Additionally, OTUD3 acts as a negative regulator of the ribosome quality control (RQC) by deubiquitinating ribosomal proteins RPS10/eS10 and RPS20/uS10, counteracting ZNF598-mediated 40S ubiquitination.

OTUD3 is also known as DUBA4.

Associated Diseases



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