title: Irradiated Blm-deficient mice are a highly tumor prone model for analysis of a broad spectrum of hematologic malignancies.
abstract: Mutations in the BLM gene cause human Bloom syndrome (BS), an autosomal recessive disorder of growth retardation, immunodeficiency and cancer predisposition. Homozygous null Blm(m3/m3) mice are cancer prone with a 5-fold increased risk of cancer compared with Blm(m3/+) and Blm(+/+) mice. Irradiation of Blm(m3/m3) mice increased the risk to 28-fold. Tumors occurred mainly in the hematopoietic system and were similar to those in BS based on detailed histologic and immunohistochemical analyses. Irradiated Blm-deficient mice thus provide a novel model for understanding accelerated malignancies in BS and a new platform for investigating the molecular basis for a wide range of hematopoietic neoplasms.
authors.affiliation: Wellcome Trust Sanger Institute, Wellcome Trust Genome Campus, Hinxton, Cambridge CB10 1SA, UK. mvw@pathologydiagnostics.com
authors.forename: Madhuri
authors.initials: M
authors.lastname: Warren
authors.forename: Yeun-Jun
authors.initials: YJ
authors.lastname: Chung
authors.forename: William J
authors.initials: WJ
authors.lastname: Howat
authors.forename: Hannah
authors.initials: H
authors.lastname: Harrison
authors.forename: Ralph
authors.initials: R
authors.lastname: McGinnis
authors.forename: Xingpei
authors.initials: X
authors.lastname: Hao
authors.forename: John
authors.initials: J
authors.lastname: McCafferty
authors.forename: Torgny N
authors.initials: TN
authors.lastname: Fredrickson
authors.forename: Allan
authors.initials: A
authors.lastname: Bradley
authors.forename: Herbert C
authors.initials: HC
authors.lastname: Morse
mesh_terms: D000818:Animals; D001816:Bloom Syndrome / Q000150:complications; D004195:Disease Models, Animal; D019337:Hematologic Neoplasms / Q000235:genetics / Q000473:pathology*; D051379:Mice; D018345:Mice, Knockout; D053484:RecQ Helicases / Q000172:deficiency*
chemical_list: C097151:Bloom syndrome protein; D053484:RecQ Helicases