Results 231 to 240 of about 51,393 (297)

A Practice Framework for Genetic Testing in Asymptomatic Relatives of Patients With Creutzfeldt‐Jakob Disease: Experience and Insights From Israel

European Journal of Neurology, Volume 33, Issue 6, June 2026.
Presymptomatic genetic testing for genetic Creutzfeldt‐Jakob disease requires careful balancing of autonomy, psychological preparedness, and potential familial implications. Based on multidisciplinary experience in Israel, we propose a structured framework including pre‐test counseling, psychological appraisal, genetic testing, in‐person results ...
Dror Shir, Noa Bregman, Aya Bar David, Talya Nathan, Orly Goldstein, Tanya Gurevich, Avner Thaler, Anat Mirelman, Tamara Shiner, Mali Gana Weisz, Shiri Shkedi‐Rafid, Gael Ganz, Hagit Shani, Elon Pras, Odelia Chorin, Gia Sorkin, Keren Markus‐Bustani, Shlomzion Kahana Merhavi, Mor Shechory‐Stahl, Miri Yanoov Sharav, Michal Dicastro, Dorin Trigubov, Dalit Barel, Esther Kahana, Avi Fellner, Amos Korczyn, Hagit Baris Feldman, Lior Greenbaum, Nurit Omer   +28 more
wiley   +1 more source

Expression of Concern: Snord 3A: A Molecular Marker and Modulator of Prion Disease Progression. [PDF]

PLoS One
PLOS ONE Editors.
europepmc   +1 more source

Redox environment modulates aggregation of ataxin‐3 in vitro — Implications for drug screening of cysteine‐rich proteins

The FEBS Journal, Volume 293, Issue 11, Page 3376-3398, June 2026.
Redox environment modulates in vitro aggregation of Ataxin‐3, the protein implicated in spinocerebellar ataxia type 3. Reducing conditions stabilize native monomers and prevent aggregation, whereas oxidative conditions promote the formation of non‐native conformers and disulfide‐linked oligomers within the Josephin domain (JD).
Martyna Podlasiak, Martina Sollazzo, Elisa Monaca, Raffaele Sabbatella, Maria Agnese Morando, Oleg Chertkov, Laura Puglisi, Martina Mascellino, Anna Fricano, Sandra Macedo‐Ribeiro, Caterina Alfano   +10 more
wiley   +1 more source

Inherited Prion Disease with a 5-octapeptide Repeat Insertion in the PRNP Gene Presenting with Familial Juvenile Dementia. [PDF]

Intern Med
Nishikawa M, Takeda A, Miyazawa N, Hara N, Ishii K, Ikeuchi T, Itoh Y.   +6 more
europepmc   +1 more source

Investigating Disordered Eating Behaviours Among Individuals Living With Neurodegenerative Disease: A Scoping Review

Journal of Human Nutrition and Dietetics, Volume 39, Issue 3, June 2026.
ABSTRACT Aim Neurodegenerative diseases and disordered eating have become rapidly expanding areas of research. However, research addressing the relationship between the two is lacking. Methods A scoping review guided by the Joanna Briggs Institute methodological framework was completed to synthesise the evidence related to disordered eating behaviours ...
Emily Buttifant, Shoroog Allogmanny, Yasmine Probst   +2 more
wiley   +1 more source

In situ assessment of neuroinflammatory cytokines in different stages of ovine natural prion disease. [PDF]

Front Vet Sci
Guijarro IM, Garcés M, Badiola JJ, Monzón M.   +3 more
europepmc   +1 more source

Effects of Lysine Deacetylation Inhibition Alone or in Combination With Arimoclomol on TDP‐43 Proteinopathy

Journal of Neurochemistry, Volume 170, Issue 6, June 2026.
TDP‐43 pathology is a hallmark of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia. Peptidyl‐prolyl cis–trans isomerase A (PPIA), a foldase and chaperone protein, modulates TDP‐43 function in an acetylation‐dependent manner. Here, we show that inhibition of lysine deacetylation with vorinostat (SAHA) increases PPIA acetylation and ...
Serena Scozzari, Stefano Fabrizio Columbro, Monica Favagrossa, Massimo Tortarolo, Alfredo Cagnotto, Mario Salmona, Giovanni De Marco, Caterina Bendotti, Andrea Calvo, Laura Pasetto, Valentina Bonetto   +10 more
wiley   +1 more source

GPI-anchorless prion disease is sensitive to oxidative stress and shows potential for treatment with edaravone, based on iPS-derived neuron study. [PDF]

Cell Mol Life Sci
Matsuzono K, Honda H, Mashiko T, Koide R, Sakashita E, Endo H, Kitamoto T, Fujimoto S.   +7 more
europepmc   +1 more source

Understanding the Phenotypic Heterogeneity Within the Sporadic Creutzfeldt–Jakob Disease MV1 Subtype

Neuropathology and Applied Neurobiology, Volume 52, Issue 3, June 2026.
We present the clinical, pathological and prion biophysical features of three atypical cases of the MV1 subtype of sporadic Creutzfeldt–Jakob disease (sCJD). We propose that these heterozygous cases exist on a spectrum ranging from MM1‐like to VV1‐like phenotypes and recommend that subtyping be performed at pH 6.9 to avoid missing atypical or mixed ...
Satish K. Nemani, Leonardo M. Cortez, Jennifer Myskiw, Ignazio Cali, Stephanie A. Booth, Gerard H. Jansen, Valerie L. Sim   +6 more
wiley   +1 more source