Herbert Budka

TL;DR: The results indicate that effects in the ELS correlate with regional pathology, and overloading of this system might impair the function of lysosomal enzymes and thus may mimic some features of l Lysosomal storage disorders.

TL;DR: Results of modern staining techniques including anti‐prion protein (PrP) immunocytochemistry to a set of archival brain specimens of a 16 year‐old male who died from kuru in 1967 are reported.

TL;DR: The results suggest that rigorous methodology and dichotomized assessment should be applied and that semiquantitative assessment can be recommended only for the cortical samples, and that there are limitations in the scoring of &agr;S-IR structures.

TL;DR: The researchers concentrated their neurological investigations on patients with acrodermatitis chronica atrophicans (ACA), which is a chronic skin disorder with established borrelial etiology, however, in these patients no C N S involvement was detected and a significant number of patients with peripheral neuropathy were seen.

TL;DR: This work presents the first effective mouse model for E200KCJD, which expresses chimeric mouse/human E199KPrP on both a null and a wt PrP background, as is the case for heterozygous patients and carriers, and believes that these mice may serve as an ideal platform for the investigation of the pathogenesis of genetic prion disease and thus for the monitoring of anti-prion treatments.