USP1


Description

The USP1 (ubiquitin specific peptidase 1) is a protein-coding gene located on chromosome 1.

USP1 is a human gene that encodes a deubiquitinating enzyme (DUB) belonging to the ubiquitin-specific processing (UBP) family of proteases. It contains His and Cys domains and is located in the cytoplasm. USP1 removes ubiquitin tags from proteins, playing a vital role in regulating protein function and degradation. It specifically deubiquitinates FANCD2, a protein involved in DNA repair, and is also involved in translesion synthesis by deubiquitinating PCNA. Multiple splice variants of USP1 have been identified.

USP1 is a negative regulator of DNA damage repair that specifically deubiquitinates monoubiquitinated FANCD2, a key protein in the Fanconi anemia pathway. USP1 is also involved in PCNA-mediated translesion synthesis (TLS) by deubiquitinating monoubiquitinated PCNA, which allows DNA replication to proceed past damaged DNA. USP1 has very limited deubiquitinating activity on its own and requires an interaction with WDR48 to become fully functional.

USP1 is also known as UBP.

Associated Diseases



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