Evidence for early cytotoxic aggregates in transgenic mice for human transthyretin Leu55Pro.

@article{Sousa2002EvidenceFE,
  title={Evidence for early cytotoxic aggregates in transgenic mice for human transthyretin Leu55Pro.},
  author={M{\'o}nica Mendes Sousa and Rui D V Fernandes and Joana Almeida Palha and Ana Taboada and Paulo Vieira and Maria Jo{\~a}o Saraiva},
  journal={The American journal of pathology},
  year={2002},
  volume={161 5},
  pages={1935-48}
}
Familial amyloidotic polyneuropathy (FAP) is a lethal autosomal dominant disorder characterized by systemic extracellular deposition of transthyretin (TTR) amyloid fibrils. Several groups have generated transgenic mice carrying human TTR Val30Met, the most common mutation in FAP. To study amyloidogenicity and cytotoxicity of different TTRs, we produced transgenic mice expressing human TTR Leu55Pro, one of the most aggressive FAP-related mutations. TTR deposition and presence of amyloid fibrils… CONTINUE READING
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