The role of SMARCAL1 in replication fork stability and telomere maintenance

Natalia Lugli; Sotirios K Sotiriou; Thanos D Halazonetis

doi:10.1016/j.dnarep.2017.06.015

The role of SMARCAL1 in replication fork stability and telomere maintenance

DNA Repair (Amst). 2017 Aug:56:129-134. doi: 10.1016/j.dnarep.2017.06.015. Epub 2017 Jun 10.

Authors

Natalia Lugli¹, Sotirios K Sotiriou¹, Thanos D Halazonetis²

Affiliations

¹ Department of Molecular Biology, University of Geneva, Switzerland.
² Department of Molecular Biology, University of Geneva, Switzerland. Electronic address: Thanos.Halazonetis@unige.ch.

PMID: 28623093
DOI: 10.1016/j.dnarep.2017.06.015

Abstract

SMARCAL1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily A-Like 1), also known as HARP, is an ATP-dependent annealing helicase that stabilizes replication forks during DNA damage. Mutations in this gene are the cause of Schimke immune-osseous dysplasia (SIOD), an autosomal recessive disorder characterized by T-cell immunodeficiency and growth dysfunctions. In this review, we summarize the main roles of SMARCAL1 in DNA repair, telomere maintenance and replication fork stability in response to DNA replication stress.

Keywords: DNA replication; DNA replication stress; SMARCAL1; Telomeres.

Publication types

Review
Research Support, Non-U.S. Gov't

MeSH terms

Animals
DNA Helicases / metabolism*
DNA Repair
DNA Replication*
Humans
Telomere / metabolism*

Substances

SMARCAL1 protein, human
Smarcal1 protein, mouse
DNA Helicases