Open AccessModeling Familial British Dementia in Transgenic Mice
Author(s) -
Pickford Fiona,
Coomaraswamy Janaky,
Jucker Mathias,
McGowan Eileen
Publication year - 2006
Publication title -
brain pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.986
H-Index - 132
eISSN - 1750-3639
pISSN - 1015-6305
DOI - 10.1111/j.1750-3639.2006.tb00564.x
Subject(s) - genetically modified mouse , dementia , transgene , gene , mutation , amyloidosis , amyloid (mycology) , biology , genetics , medicine , cancer research , pathology , bioinformatics , disease
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.