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USP51

Function

Specifically deubiquitinates 'Lys-14' (H2AK13Ub) and 'Lys-16'(H2AK15Ub) of histone H2A regulating the DNA damage response at double-strand breaks (DSBs) (PubMed:27083998, PubMed:33022275). USP51 is recruited to chromatin after DNA damage and regulates the dynamic assembly/disassembly of TP53BP1 and BRCA1. Functions in DNA double-strand break repair also by mediating the deubiquitination and subsequent stabilization of DGCR8, leading to the recruitment of DGCR8 binding partners to double strand breaks such as RNF168 or MDC1 (PubMed:34188037). In addition, promotes the deubiquitination and stabilization of the transcriptional repressor ZEB1 (PubMed:29119051).

Sequence Similarities

Belongs to the peptidase C19 family.

Tissue Specificity

Expressed in prostate, brain, lung, aorta and kidney.

Cellular localization

Alternative names

Ubiquitin carboxyl-terminal hydrolase 51, Deubiquitinating enzyme 51, Ubiquitin thioesterase 51, Ubiquitin-specific-processing protease 51, USP51

swissprot:Q70EK9 entrezGene:158880