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.