[HTML][HTML] Crystallographic studies of prion protein (PrP) segments suggest how structural changes encoded by polymorphism at residue 129 modulate susceptibility to …

MI Apostol, MR Sawaya, D Cascio… - Journal of biological …, 2010 - ASBMB
A single nucleotide polymorphism (SNP) in codon 129 of the human prion gene, leading to a
change from methionine to valine at residue 129 of prion protein (PrP), has been shown to
be a determinant in the susceptibility to prion disease. However, the molecular basis of this
effect remains unexplained. In the current study, we determined crystal structures of prion
segments having either Met or Val at residue 129. These 6-residue segments of PrP
centered on residue 129 are" steric zippers," pairs of interacting β-sheets. Both structures of …