{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:49:08Z","timestamp":1791298148660,"version":"4.1.0"},"reference-count":54,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2011,11,14]],"date-time":"2011-11-14T00:00:00Z","timestamp":1321228800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["pathsocjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Pathology"],"published-print":{"date-parts":[[2012,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Fanconi anaemia (FA) is a rare recessive disorder marked by developmental abnormalities, bone marrow failure, and a high risk for the development of leukaemia and solid tumours. The inactivation of FA genes, in particular <jats:italic>FANCF<\/jats:italic>, has also been documented in sporadic tumours in non\u2010FA patients. To study whether there is a causal relationship between FA pathway defects and tumour development, we have generated a mouse model with a targeted disruption of the FA core complex gene <jats:italic>Fancf. Fancf<\/jats:italic>\u2010deficient mouse embryonic fibroblasts displayed a phenotype typical for FA cells: they showed an aberrant response to DNA cross\u2010linking agents as manifested by G<jats:sub>2<\/jats:sub> arrest, chromosomal aberrations, reduced survival, and an inability to monoubiquitinate FANCD2. <jats:italic>Fancf<\/jats:italic> homozygous mice were viable, born following a normal Mendelian distribution, and showed no growth retardation or developmental abnormalities. The gonads of <jats:italic>Fancf<\/jats:italic> mutant mice functioned abnormally, showing compromised follicle development and spermatogenesis as has been observed in other FA mouse models and in FA patients. In a cohort of <jats:italic>Fancf<\/jats:italic>\u2010deficient mice, we observed decreased overall survival and increased tumour incidence. Notably, in seven female mice, six ovarian tumours developed: five granulosa cell tumours and one luteoma. One mouse had developed tumours in both ovaries. High\u2010resolution array comparative genomic hybridization (aCGH) on these tumours suggests that the increased incidence of ovarian tumours correlates with the infertility in <jats:italic>Fancf<\/jats:italic>\u2010deficient mice and the genomic instability characteristic of FA pathway deficiency. Copyright \u00a9 2011 Pathological Society of Great Britain and Ireland. Published by John Wiley &amp; Sons, Ltd.<\/jats:p>","DOI":"10.1002\/path.2992","type":"journal-article","created":{"date-parts":[[2011,9,7]],"date-time":"2011-09-07T05:49:32Z","timestamp":1315374572000},"page":"28-39","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["<i>Fancf<\/i>\u2010deficient mice are prone to develop ovarian tumours"],"prefix":"10.1002","volume":"226","author":[{"given":"Sietske T","family":"Bakker","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henri J","family":"van de Vrugt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jenny 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