Results 81 to 90 of about 35,863 (248)

Gerstmann-Sträussler-Scheinker disease revisited: accumulation of covalently-linked multimers of internal prion protein fragments [PDF]

, 2019
Despite their phenotypic heterogeneity, most human prion diseases belong to two broadly defined groups: Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker disease (GSS).
Cali, Ignazio, Cracco, Laura, Gambetti, Pierluigi, Ghetti, Bernardino, Lavrich, Jody, Nemani, Satish K., Notari, Silvio, Surewicz, Witold K., Xiao, Xiangzhu   +8 more
core   +1 more source

Evidence for different seeding activities of misfolded tau in classical and rapidly progressive Alzheimer's disease

Brain Pathology, EarlyView.
The study analyzed the seeding activity of misfolded tau protein in Alzheimer's disease and rapidly progressive Alzheimer's disease patients and provides evidence for the existence of different tau assemblies supported by differences in cellular toxicity and morphology of thioflavin T‐positive real‐time quaking‐induced conversion products.
Matthias Schmitz, Katrin Thüne, Anna‐Lisa Fischer, Peter Hermann, Maria Cramm, Leonie Sheila Kadel, Wiebke Möbius, Jakob Matschke, Tayyaba Saleem, Neelam Younas, Saima Zafar, Hermann Altmeppen, Markus Glatzel, Inga Zerr   +13 more
wiley   +1 more source

Creutzfeldt-Jakob disease: recent developments [PDF]

F1000Research, 2017
Creutzfeldt-Jakob disease (CJD) is a rare prion disorder that has been the subject of both professional and public interest following the identification of variant CJD as a zoonotic disorder. There have been recent advances in diagnostic techniques, including real-time quaking-induced conversion and magnetic resonance imaging brain scan, that have ...
Graeme Mackenzie, Robert Will
openaire   +3 more sources

Rapid generation of prion disease models using AAV‐delivered PrP variants in knockout mice

Brain Pathology, EarlyView.
We developed a rapid AAV‐based system to generate prion disease models in weeks rather than months. Following systemic AAV9P31 delivery of modified PrP to knockout mice, we achieved brain‐wide expression and successful propagation of both classical (RML) and atypical (GSS‐A117V) prion strains.
Maitena San‐Juan‐Ansoleaga, Eva Fernández‐Muñoz, Jorge M. Charco, Enric Vidal, Diego Herrero‐Martínez, Josu Galarza‐Ahumada, Cristina Sampedro‐Torres‐Quevedo, Samanta Giler, Mariví Geijo, Gloria González‐Aseguinolaza, Hasier Eraña, Joaquín Castilla   +11 more
wiley   +1 more source

Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding [PDF]

, 2010
Transmissible spongiform encephalopathies, or prion diseases, are caused by misfolding and aggregation of the prion protein PrP. These diseases can be hereditary in humans and four of the many disease-associated missense mutants of PrP are in the ...
Daggett, Valerie, van der Kamp, Marc W
core   +2 more sources

Implant Therapy in Patients With Neurodegenerative Diseases—A Scoping Review

Clinical Oral Implants Research, EarlyView.
ABSTRACT Objectives The increasing longevity of populations has resulted in a growing number of older adults requiring prosthodontic care, including those with neurodegenerative diseases (NDs). Neurodegenerative diseases pose significant challenges to prosthodontic care, and it remains unclear whether implant therapy in this population achieves ...
Lysandre David, Sabrina Maniewicz, Najla Chebib, Gabriel Gold, Murali Srinivasan, Frauke Müller   +5 more
wiley   +1 more source

Sporadic Creutzfeldt–Jakob Disease

Acta Medica Bulgarica
Sporadic Creutzfeldt–Jakob disease is a rare and fatal human prion disease characterized by a rapidly progressive dementia, myoclonus, cerebellar, pyramidal, extrapyramidal, visual, and psychiatric symptoms. These findings are all non-specific and making
Zhelyazkova S., Nachev S., Kalev O., Tournev I.   +3 more
doaj   +1 more source

Probable sporadic Creutzfeldt–Jakob disease mimicking focal epilepsy

Epilepsy and Behavior Case Reports, 2019
Creutzfeldt–Jakob disease (CJD) presents with seizures as an early symptom in only approximately 3% of cases. These seizures often present as nonconvulsive status epilepticus (NCSE) or epilepsia partialis continua (EPC).
Pei-Shan Hsiao, Yuan-Ming Lee, Fu-Sin Chu, Chao-Lin Lee, Fang-Chun Liu, Ping-Huang Tsai   +5 more
doaj   +1 more source

Intra- and interspecies interactions between prion proteins and effects of mutations and polymorphisms [PDF]

, 2003
Recently, crystallization of the prion protein in a dimeric form was reported. Here we show that native soluble homogenous FLAG-tagged prion proteins from hamster, man and cattle expressed in the baculovirus system are predominantly dimeric.
Bradley R., Campbell T.A., Clark W.W., Cutlip R.C., Edenhofer F., Goldfarb L.G., Krasemann S., Leucht C., Lopez L., Majtenyi C., Scott M.R., Weiss S.   +11 more
core   +1 more source

Epidemiology of progressive intellectual and neurological deterioration in UK children

Developmental Medicine &Child Neurology, EarlyView.
This study of PIND in UK children was carried out via the British Paediatric Surveillance Unit from 1997 to 2024. It identified six cases of vCJD. 2367 children had other diagnoses explain their deterioration. There were 259 other diseases in the diagnosed group.
Christopher M. Verity, Polly J. Maunder, Anne Marie Winstone, Suvankar Pal   +3 more
wiley   +1 more source