title: Modeling familial British dementia in transgenic mice.
abstract: The chromosome 13 linked amyloidopathies familial British dementia (FBD) and familial Danish dementia (FDD) are caused by mutations in the C-terminus of the BRI2 gene. In both diseases, novel peptides are deposited in amyloid plaques in the brain. Several laboratories have attempted to model these diseases in BRI2 transgenic mice with limited success. While high expression levels of BRI protein were achieved in transgenic lines, no ABri-amyloidosis was observed in aged mice. This review discusses the strategies chosen and problems experienced with the development of FBD/FDD models and suggests novel approaches to model the diseases in murine models.
authors.affiliation: Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL 92224, USA.
authors.forename: Fiona
authors.initials: F
authors.lastname: Pickford
authors.forename: Janaky
authors.initials: J
authors.lastname: Coomaraswamy
authors.forename: Mathias
authors.initials: M
authors.lastname: Jucker
authors.forename: Eileen
authors.initials: E
authors.lastname: McGowan
mesh_terms: D048868:Adaptor Proteins, Signal Transducing; D000818:Animals; D016657:Cerebral Amyloid Angiopathy / Q000235:genetics* / Q000473:pathology*; D003704:Dementia / Q000235:genetics* / Q000473:pathology*; D045683:Furin / Q000096:biosynthesis / Q000235:genetics; D006801:Humans; D008565:Membrane Proteins / Q000235:genetics / Q000378:metabolism; D051379:Mice; D008822:Mice, Transgenic
chemical_list: D048868:Adaptor Proteins, Signal Transducing; C492300:Itm2b protein, mouse; D008565:Membrane Proteins; D045683:Furin