Progress of WRAP53 beta Regulating DNA Damage Repair

WRAP53 beta, a protein with a WD40 domain, plays an essential role in the maintenance of Cajal body stability, RNA splicing, and telomere elongation. WRAP53 beta dysfunction is related to dyskeratosis congenita, tumor, progressive spinal muscular atrophy, premature aging and other diseases. Recent studies have found that WRAP53 beta is an important scaffold protein essential for DNA double strand break repair (DSBs). It can be recruited to DNA damage sites in a H2AX, ATM and MDC1-dependent manner and phosphorylated by ATM. Then the phosphorylated WRAP53 beta recruits ubiquitin E3 ligase RNF8 through its WD40 domain leading to histone H2AX ubiquitination, and further aggregation of downstream repair factors for DNA damage repair. In this review, we summarize the recent advances in the specific role and underlying mechanisms of WRAP53 beta in the repair of DNA damages.