1. Modeling Familial British Dementia in Transgenic Mice
- Author
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Mathias Jucker, Eileen McGowan, Janaky Coomaraswamy, and Fiona Pickford
- Subjects
Genetically modified mouse ,Transgene ,Mice, Transgenic ,Pathology and Forensic Medicine ,Mice ,Transgenic lines ,Animals ,Humans ,Medicine ,Dementia ,Gene ,Adaptor Proteins, Signal Transducing ,FAMILIAL DANISH DEMENTIA ,Chromosome 13 ,Furin ,Genetics ,business.industry ,Extramural ,General Neuroscience ,Membrane Proteins ,medicine.disease ,Cerebral Amyloid Angiopathy ,SYMPOSIUM: Cerebral Amyloid Angiopathies ,Neurology (clinical) ,business - 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.
- Published
- 2006
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