title: [Fanconi anemia animal models - How differences can teach us as much as similarities…].
abstract: Fanconi Anemia is a rare autosomal recessive genetic disease with heterogenous phenotypes including myelosuppression, congenital malformations and heightened cancer predisposition. FA cells are highly sensitive to cross-linking agents. Since the 90's, at least 19 FANC proteins (FANCA to FANCT) have been identified as working together in a unique pathway detecting and triggering the repair of DNA crosslinks. Since then, the creation of animal models in various species (nematode, fruit fly, zebrafish and mouse) contributed to a better understanding of the physiopathology of the disease. This review aims to summarize the main discoveries made in these in vivo models, as well as to discuss some controversies that arose from these studies.
authors.affiliation: Département de biologie moléculaire, biochimie médicale, et pathologie et Centre de Recherche sur le Cancer, Université Laval, Canada - CRCHU de Québec, axe oncologie, 9 McMahon, Québec, QC, G1R 3S3, Canada.
authors.forename: Émilie L
authors.initials: ÉL
authors.lastname: Dubois
authors.affiliation: Département de biologie moléculaire, biochimie médicale, et pathologie et Centre de Recherche sur le Cancer, Université Laval, Canada - CRCHU de Québec, axe oncologie, 9 McMahon, Québec, QC, G1R 3S3, Canada.
authors.forename: Mariline
authors.initials: M
authors.lastname: Béliveau
authors.affiliation: Département de biologie moléculaire, biochimie médicale, et pathologie et Centre de Recherche sur le Cancer, Université Laval, Canada - CRCHU de Québec, axe oncologie, 9 McMahon, Québec, QC, G1R 3S3, Canada.
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Masson
mesh_terms: D000818:Animals; D017173:Caenorhabditis elegans; D004195:Disease Models, Animal; D004331:Drosophila melanogaster; D005199:Fanconi Anemia / Q000235:genetics / Q000473:pathology*; D051856:Fanconi Anemia Complementation Group Proteins / Q000235:genetics; D006801:Humans; D051379:Mice; D015027:Zebrafish
chemical_list: D051856:Fanconi Anemia Complementation Group Proteins