3 Apr 2014 Past and future problems surrouding dura mater graft-associated Creutzfeldt-Jakob
disease (dCJD)― dCJD can be divided into two subgroups that exhibit distinct clinical
and neuropathological features: non-plaque-type and plaque-type. Recent
progress in research of the pathogenesis of dCJD has revealed that two
distinct subgroups of dCJD are caused by infection with different prion
strains from sCJD. These studies have also revealed previously unrecognized
problems as follows: (i) the numbers of patients with plaque-type dCJD
may increase in the future, (ii) the potential risks of secondary infection
from dCJD, particularly from plaque-type, may be considerable, and (iii)
the effectiveness of the current prion decontamination procedures against
plaque-type dCJD is uncertain. In our paper published in Acta Neuropathol Commun, we summarize the past and future problems surrounding dCJD.
1 Apr 2014 Registration for APPS2014 started.
20 Feb 2014 A brandnew structural model of prions― Detailed structure of the abnormal prion protein is one of the biggest
enigma remained in the field of prion research. A number of structural
models has been proposed so far, but unbiased comparison of these models
has never been performed. In our paper published in Structure, these structural models were evaluated using a comprehensive dataset
obtained from cell-based conversion assays using 2,641 mutant prion protein
constructs carrying a single amino acid substitution. Furthermore, we propose
a improved structural model of abnormal prion proteins.
13 Nov 2013 Revision of classification of sporadic Creutzfeldt-Jakob disease-MV2 (sCJD-MV2)― sCJD-MV2 has been divided into three subgroups due to its phenotypic heterogeneity. In our paper published in Acta Neuropathol Commun, a comprehensive analysis of the
sCJD-MV2 cases shows that the three subgroups of sCJD-MV2 consist of different
prion strain(s), reflecting their different origin(s). To classify this
complicated entity more adequately, we propose a refined classification
system.
21 June 2013 Unexpected susceptibility and lack of characteristic neuropathology in variant Creutzfeldt-Jakob disease (vCJD)-inoculated humanized mice carrying the PRNP genotype formerly considered as vCJD-resistant ― Recently published our paper raises a concern regarding "second wave" of vCJD infection in
individuals with the PRNP genotypes other than 129M/M, which is the sole genotype observed in clinical
vCJD cases so far. If it occurs, our animal model would be useful for the
differential diagnosis of these atypical vCJD cases.
29 May 2013 Registration for APPS2013 started.
20 May 2013 Website updated.