Apolipoprotein AII (apoAII) amyloidosis an autosomal dominant inherited form of amyloidosis caused by mutations in the stop codon for the apoAII gene is characterized by progressive renal and cardiac amyloid deposition. To study the pathogenesis of this disease, transgenic mice were developed with a DNA construct of a human amyloid associated apoAII gene. Animals expressing the transgene developed renal amyloid deposition by 3= to 4 months of age and subsequently amyloid in spleen, liver, and heart. Amyloid fibrils were extracted from tissues and biochemically shown to contain fulllength human apoAII with a 21 amino acid carboxyl terminal extension. The rapid development of amyloid deposition in these animals indicates that this will be a valuable model to study pathogenesis of amyloidosis plus afford a means of testing potential therapeutics.
ASJC Scopus subject areas
- Internal Medicine