Brain microglia were activated in sporadic CJD but almost unchanged in fatal familial insomnia and G114V genetic CJD
Brain microglia were activated in sporadic Creutzfeldt-Jakob disease, but silent in fatal familial insomnia and familial CJD, showing the various pathogenesis of different human prion diseases (Source: Virology Journal)
Source: Virology Journal - July 1, 2013 Category: Virology Authors: Qi ShiWu-Ling XieBaoYun ZhangLi-Na ChenYin XuKe WangKe RenXiao-Mei ZhangCao ChenJin ZhangXiao-Ping Dong Source Type: research
Epitope scanning indicates structural differences in brain-derived, monomeric and aggregated mutant prion proteins related to genetic prion diseases
Genetic Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia, and prion protein cerebral amyloid angiopathy are clinically and neuropathologically distinct neurodegenerative diseases linked to mutations in the PRNP gene encoding the cellular prion protein (PrPC). How sequence variants of PRNP encode the information to specify these disease phenotypes is not known. It is suggested that each mutation produces a misfolded variant of PrPC with specific neurotoxic properties. However, structural studies of recombinant PrP did not detect major differences between wild-type and mutant...
Source: BJ Disease - June 28, 2013 Category: Biochemistry Authors: L Tapella, M Stravalaci, A Bastone, E Biasini, M Gobbi, R Chiesa Tags: BJ Biomolecules Source Type: research
Molecular dynamics studies on 3D structures of the hydrophobic region PrP(109-136).
This article gives detailed reviews on the PrP(109-136) region and presents the studies of its three-dimensional structures and structural dynamics.
PMID: 23563221 [PubMed - as supplied by publisher] (Source: Acta Biochimica et Biophysica Sinica)
Source: Acta Biochimica et Biophysica Sinica - April 5, 2013 Category: Biochemistry Authors: Zhang J, Zhang Y Tags: Acta Biochim Biophys Sin (Shanghai) Source Type: research
Dream imagery, rapid eye movement sleep behavior disorder, and hallucinations
Abstract
Rapid eye movement (REM) sleep behavior disorder (RBD) and hypnagogic hallucinations are salient symptoms of abnormal and dissociated REM sleep that are frequently associated in serious neurological diseases. RBD is a strong, independent risk factor for hallucinations in narcolepsy (odds ratio: 4.3) and in Parkinson's disease (odds ratio: 2.7). In Parkinson's disease, RBD also predicts incident hallucinations and psychosis in prospective cohorts. Status dissociatus (a mixture of hallucinations, RBD, and dissociated sleep–wake states) is observed in patients with Guillain‐Barré when hallucinating, but also in ...
Source: Sleep and Biological Rhythms - March 7, 2013 Category: Neurology Authors: Isabelle ARNULF Source Type: research
