Ubiquitin specific peptidase 1, shorted as USP1, is a kind of enzyme to remove ubiquitin from proteins (DUB), which admittedly participated in regulating some pathways of damaged DNA repair, such as the Fanconi anemia (FA) pathway and translesion synthesis (TLS) process [16, 17, 18]. This evidence concerns the gene USP1 and Friedreich ataxia.