Modeling familial British dementia in transgenic mice.
Brain Pathol
; 16(1): 80-5, 2006 Jan.
Article
in En
| MEDLINE
| ID: mdl-16612985
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Cerebral Amyloid Angiopathy
/
Dementia
Limits:
Animals
/
Humans
Language:
En
Journal:
Brain Pathol
Journal subject:
CEREBRO
/
PATOLOGIA
Year:
2006
Document type:
Article
Affiliation country:
Estados Unidos