{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Luebben SW"],"funding":["NCI NIH HHS"],"pagination":["10283-97"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC3905894"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["41(22)"],"pubmed_abstract":["HELQ is a superfamily 2 DNA helicase found in archaea and metazoans. It has been implicated in processing stalled replication forks and in repairing DNA double-strand breaks and inter-strand crosslinks. Though previous studies have suggested the possibility that HELQ is involved in the Fanconi anemia (FA) pathway, a dominant mechanism for inter-strand crosslink repair in vertebrates, this connection remains elusive. Here, we investigated this question in mice using the Helq(gt) and Fancc(-) strains. Compared with Fancc(-)(/)(-) mice lacking FANCC, a component of the FA core complex, Helq(gt/gt) mice exhibited a mild of form of FA-like phenotypes including hypogonadism and cellular sensitivity to the crosslinker mitomycin C. However, unlike Fancc(-)(/)(-) primary fibroblasts, Helq(gt/gt) ce"],"journal":["Nucleic acids research"],"pubmed_title":["Helq acts in parallel to Fancc to suppress replication-associated genome instability."],"pmcid":["PMC3905894"],"funding_grant_id":["R01CA148806","P30 CA077598","R01 CA148806"],"pubmed_authors":["Akre MK","O'Sullivan MG","Shima N","Kawabata T","Johnson CS","Luebben SW","Lee WL"],"additional_accession":[]},"is_claimable":false,"name":"Helq acts in parallel to Fancc to suppress replication-associated genome instability.","description":"HELQ is a superfamily 2 DNA helicase found in archaea and metazoans. It has been implicated in processing stalled replication forks and in repairing DNA double-strand breaks and inter-strand crosslinks. Though previous studies have suggested the possibility that HELQ is involved in the Fanconi anemia (FA) pathway, a dominant mechanism for inter-strand crosslink repair in vertebrates, this connection remains elusive. Here, we investigated this question in mice using the Helq(gt) and Fancc(-) strains. Compared with Fancc(-)(/)(-) mice lacking FANCC, a component of the FA core complex, Helq(gt/gt) mice exhibited a mild of form of FA-like phenotypes including hypogonadism and cellular sensitivity to the crosslinker mitomycin C. However, unlike Fancc(-)(/)(-) primary fibroblasts, Helq(gt/gt) ce","dates":{"release":"2013-01-01T00:00:00Z","publication":"2013 Dec","modification":"2025-04-22T19:28:36.47Z","creation":"2019-03-27T01:20:51Z"},"accession":"S-EPMC3905894","cross_references":{"pubmed":["24005041"],"doi":["10.1093/nar/gkt676"]}}