Emerging roles of DNA repair factors in the stability of centromeres

Satellite DNA sequences are an integral part of centromeres, regions critical for faithful segregation of chro-mosomes during cell division. Because of their complex repetitive structure, satellite DNA may act as a barrier to DNA replication and other DNA based transactions ultimately resulting in chromosome breakage. Over the past two decades, several DNA repair proteins have been shown to bind and function at centromeres. While the importance of these repair factors is highlighted by various structural and numerical chromosome aberrations resulting from their inactivation, their roles in helping to maintain genome stability by solving the intrinsic difficulties of satellite DNA replication or promoting their repair are just starting to emerge. In this review, we summarize the current knowledge on the role of DNA repair and DNA damage response proteins in maintaining the structure and function of centromeres in different contexts. We also report the recent connection between the roles of specific DNA repair factors at these genomic loci with age-related increase of chromosomal instability under physiological and pathological conditions.

Automated summary

Satellite DNA sequences are a crucial part of centromeres, which are essential for the accurate segregation of chromosomes during cell division. Due to their complex repetitive structure, satellite DNA can hinder DNA replication and other DNA-based processes, potentially leading to chromosome breakage. In recent years, several DNA repair proteins have been found to interact with and function at centromeres, contributing to the maintenance of their structure and function. While the importance of these repair factors has been demonstrated by chromosome aberrations resulting from their inactivation, their roles in satellite DNA replication and repair are still being explored. Recent studies have also linked specific DNA repair factors at centromeres to age-related increase in chromosomal instability under physiological and pathological conditions.