Results 81 to 90 of about 9,411 (168)

Potential genetic robustness of Prnp and Sprn double knockout mouse embryos towards ShRNA-lentiviral inoculation

open access: yes, 2021
Shadoo, encoded by Sprn , and PrP, encoded by Prnp , are related proteins whose biological functions are still incompletely understood. Although previous knockdown experiments have suggested the necessity of Shadoo in the absence of PrP during early ...
Daniel-Carlier, Nathalie   +10 more
core   +1 more source

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

open access: yesBrain Pathology, Volume 36, Issue 4, July 2026.
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   +11 more
wiley   +1 more source

The Effects of the PRNP Gene on Circadian Rhythms

open access: yes, 2014
Fatal Familial Insomnia is a rare genetic disorder affecting about 27 families worldwide, caused by mutations in the prion protein gene PRNP. Symptoms include loss of sleep and disrupted circadian rhythms. The function of PNRP in circadian rhythms is not
Vlasac, Irma Marisela
core   +3 more sources

Molecular Characterization of the Rocky Mountain Elk (Cervus elaphus nelsoni) PRNP Putative Promoter [PDF]

open access: yes, 2007
Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) affecting deer (Odocoileus spp.), moose (Alces alces), and Rocky Mountain elk (Cervus elaphus nelsoni).
Derr, J.   +9 more
core   +2 more sources

The L108I polymorphism in mouse prion protein drives spontaneous disease and enhances transmission of atypical and classical prion strains

open access: yesBrain Pathology, Volume 36, Issue 4, July 2026.
A single amino acid change (L108I) combined with PrP overexpression drives spontaneous atypical prion formation in mice, enabling also efficient propagation of diverse prion strains. This model allows studying how spontaneous prion diseases arise and provides powerful tools for investigating strain emergence, transmission barriers, and mechanisms ...
Hasier Eraña   +20 more
wiley   +1 more source

Genetic characterization of the prion protein gene in camels (Camelus) with comments on the evolutionary history of prion disease in Cetartiodactyla [PDF]

open access: yesPeerJ
Transmissible spongiform encephalopathies (TSEs) are a fatal neurogenerative disease that include Creutzfeldt–Jakob disease in humans, scrapie in sheep and goats, bovine spongiform encephalopathy (BSE), and several others as well as the recently ...
Emily A. Wright   +5 more
doaj   +2 more sources

Lack of association of the M129V polymorphism of the PRNP gene with pseudoexfoliation syndrome

open access: yes, 2016
Marios P Giannakopoulos,1 Anna G Antonacopoulou,2 Anastasia E Kottorou,2 Haralabos P Kalofonos,2 Sotirios P Gartaganis1 1Department of Ophthalmology, School of Medicine, 2Department of Medicine, Molecular Oncology Laboratory, Division of Oncology ...
Kalofonos HP   +4 more
core  

Identification of two novel mutations, PSEN1 E280K and PRNP G127S, in a Malaysian family

open access: yes, 2015
Gaik-Siew Ch’ng,1,* Seong Soo A An,2,* Sun Oh Bae,2 Eva Bagyinszky,2 SangYun Kim3,41Department of Genetics, Kuala Lumpur Hospital, Malaysia; 2Department of Bionano Technology, Gachon University, 3Department of Neurology, Seoul National ...
Kim SY   +4 more
core  

Cultured Prnp0/0 oligodendrocytes remain in an undifferentiated state for longer than the Prnp+/+ controls.

open access: yes, 2012
A) Low power photomicrograph of a primary Prnp+/+ mixed culture showing NG2-positive oligodendrocytes (arrows) grown and differentiated over a GFAP-positive astrocyte monolayer.
Rosalina Gavín (171289)   +8 more
core   +1 more source

Ovine reference materials and assays for prion genetic testing

open access: yesBMC Veterinary Research, 2010
Background Genetic predisposition to scrapie in sheep is associated with several variations in the peptide sequence of the prion protein gene (PRNP). DNA-based tests for scoring PRNP codons are essential tools for eradicating scrapie and for evaluating ...
Smith Timothy PL   +6 more
doaj   +1 more source

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