Brain Structure and Function

, Volume 214, Issue 2–3, pp 235–244

Modeling familial British and Danish dementia

  • Holly J. Garringer
  • Jill Murrell
  • Luciano D’Adamio
  • Bernardino Ghetti
  • Ruben Vidal
Review

DOI: 10.1007/s00429-009-0221-9

Cite this article as:
Garringer, H.J., Murrell, J., D’Adamio, L. et al. Brain Struct Funct (2010) 214: 235. doi:10.1007/s00429-009-0221-9

Abstract

Familial British dementia (FBD) and familial Danish dementia (FDD) are two autosomal dominant neurodegenerative diseases caused by mutations in the BRI2 gene. FBD and FDD are characterized by widespread cerebral amyloid angiopathy (CAA), parenchymal amyloid deposition, and neurofibrillary tangles. Transgenic mice expressing wild-type and mutant forms of the BRI2 protein, Bri2 knock-in mutant mice, and Bri2 gene knock-out mice have been developed. Transgenic mice expressing a human FDD-mutated form of the BRI2 gene have partially reproduced the neuropathological lesions observed in FDD. These mice develop extensive CAA, parenchymal amyloid deposition, and neuroinflammation in the central nervous system. These animal models allow the study of the molecular mechanism(s) underlying the neuronal dysfunction in these diseases and allow the development of potential therapeutic approaches for these and related neurodegenerative conditions. In this review, a comprehensive account of the advances in the development of animal models for FBD and FDD and of their relevance to the study of Alzheimer disease is presented.

Keywords

FBD FDD Mouse models Neurodegeneration Amyloid CAA Neuroinflammation 

Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Holly J. Garringer
    • 1
  • Jill Murrell
    • 1
  • Luciano D’Adamio
    • 2
  • Bernardino Ghetti
    • 1
  • Ruben Vidal
    • 1
  1. 1.Department of Pathology and Laboratory Medicine, Indiana Alzheimer Disease CenterIndiana University School of MedicineIndianapolisUSA
  2. 2.Department of Microbiology and ImmunologyAlbert Einstein College of MedicineNYUSA

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