Posted 27 May 2008
Transgene: 10-nucleotide duplication (TTTAATTTGT) was introduced in the BRI2 complementary
DNA (cDNA) sequence by PCR, and the resulting BRI2795InsTTTAATTTGT cDNA sequence
was PCR-amplified using oligonucleotide primers containing an XhoI site, a Kozak
consensus sequence and a stop codon. The BRI2795InsTTT AATTTGT cDNA was inserted
into the pBS/MoPrP.Xho vector and microinjected into hybrid C3HeB/FeJ mouse embryos.
Mutation: Familial Danish dementia (FDD) form of BRI2
Promoter: Mouse prion protein promoter
Mouse strain: Hybrid C3HeB/FeJ mouse embryos
Background strain: Background C57BL/6J, 10 generations
Colony Maintenance: Cross transgenic animals to non-transgenic C57BL/6J
Widespread cerebral amyloid angiopathy (CAA) (~7 months), parenchymal ADan deposition,
vascular amyloid deposition, amyloid associated gliosis, intracellular and extracellular
deposition of oligomeric forms of ADan, tau immunoreactive deposits in neuropil.
Aged (~1year) animals show abnormal grooming behavior, an arched back and walk with
a wide-based gait and shorter steps.
Contact: Ruben Vidal, Dept of Pathology and Laboratory Medicine, Indiana Univ. School
of Med, 635 Barnhill Drive MSB A136, Indianapolis, IN 46202, Phone: (317) 274-1729,
Fax: (317) 278-6613, E-mail: firstname.lastname@example.org
Vidal R, Barbeito AG, Miravalle L, Ghetti B. Cerebral Amyloid Angiopathy and Parenchymal
Amyloid Deposition in Transgenic Mice Expressing the Danish Mutant Form of Human
BRI(2). Brain Pathol. 2008 Apr 10; Abstract