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Publication : Modeling familial British dementia in transgenic mice.

First Author  Pickford F Year  2006
Journal  Brain Pathol Volume  16
Issue  1 Pages  80-5
PubMed ID  16612985 Mgi Jnum  J:140082
Mgi Id  MGI:3811712 Doi  10.1111/j.1750-3639.2006.tb00564.x
Citation  Pickford F, et al. (2006) Modeling familial British dementia in transgenic mice. Brain Pathol 16(1):80-5
abstractText  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.
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