NR AWIV
AU Lee,L.Y.L.; Chen,R.P.Y.
TI Evaluation of risk factor of BSE and vCJD transmission regarding human codon 129 polymorphism
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Poster sessions RA-06
PT Konferenz-Poster
AB Prion diseases comprise a group of neurodegenerative transmissible spongiform encephalopathies (TSE). The epidemic of mad cow disease in UK in 1990s and its recent spread to humans have received broad attention all over the world. Human prion gene presents a common polymorphism at codon 129, resulting in either methionine or valine. Recent studies showed that the polymorphism at codon 129 of the human prion gene has a profound effect on the susceptibility in human prion disease. In order to quantify the species barrier of prion transmission, we developed a seed titration method to evaluate it. Prion peptides corresponding to human PrP sequence 108 to 144 and bovine PrP sequence 111 to 147 were used as a model system. The seeding efficiency among bovine PrP (111-147) and human PrP (108-144) with different genotypes at codon 129 were evaluated by time-resolved Circular Dichroism (CD) spectroscopy. Our finding suggested that the individuals with methionine homozygote at codon 129 (huPrP129M) has the highest susceptibility to bovine PrP fibrils, followed by the heterozygote (huPrP129M/V), and those with valine homozygote (huPrP129V) has the lowest susceptibility. Moreover, the risk of acquiring vCJD from human was also assessed by the same peptide system. Interestingly, the risk factor of intra-species prion transmission among different human genotypes was higher than the bovine human transmission, especially for the heterozygote (huPrP129M/V) and the valine homozygote (huPrP129V).
AD Institute of Biological Chemistry, Academia Sinica, Taipei, Taiwan, ROC. E-mail: pyc@gate.sinica.edu.tw
SP englisch
PO Italien