Results 241 to 250 of about 45,736 (298)

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

Perspectives on CRISPR Genome Editing to Prevent Prion Diseases in High-Risk Individuals. [PDF]

Biomedicines
Medd MM, Cao Q.
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

Interactions between Cytokines and the Pathogenesis of Prion Diseases: Insights and Implications. [PDF]

Brain Sci
Assis-de-Lemos G, Moura-do-Nascimento R, Amaral-do-Nascimento M, Miceli AC, Vieira TCRG.   +4 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

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

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

Assessment of the therapeutic potential of Hsp70 activator against prion diseases using in vitro and in vivo models. [PDF]

Front Cell Dev Biol
Zayed M, Kim YC, Jeong BH.
europepmc   +1 more source

Large-scale validation of skin prion seeding activity as a biomarker for diagnosis of prion diseases. [PDF]

Acta Neuropathol
Zhang W, Orrú CD, Foutz A, Ding M, Yuan J, Shah SZA, Zhang J, Kotobelli K, Gerasimenko M, Gilliland T, Chen W, Tang M, Cohen M, Safar J, Xu B, Hong DJ, Cui L, Hughson AG, Schonberger LB, Tatsuoka C, Chen SG, Greenlee JJ, Wang Z, Appleby BS, Caughey B, Zou WQ.   +25 more
europepmc   +1 more source

Efficient transmission of human prion diseases to a glycan-free prion protein-expressing host. [PDF]

Brain
Cracco L, Cali I, Cohen ML, Aslam R, Notari S, Kong Q, Newell KL, Ghetti B, Appleby BS, Gambetti P.   +9 more
europepmc   +1 more source