Genetic prion diseases (gPrDs) caused by mutations in the prion protein gene (PRNP) have been classified as genetic Creutzfeldt–Jakob disease, Gerstmann–Sträussler–Scheinker disease, or …

Sep 14, 2016 · Here, we demonstrate that primary cultures of astrocytes and fibroblasts from prnp-null mice secreted lower levels of exosomes than wild-type cells. Furthermore, prnp-null …

Dec 15, 2023 · By employing highly discriminatory anti-PrP antibodies and conversion-tolerant PrP chimera, we here report that de novo PrP conversion and formation of fibril-like PrP …

Feb 28, 2022 · Prion disease is a rare, fatal, and often rapidly progressive neurodegenerative disease. Ten to fifteen percent of cases are caused by autosomal dominant gain-of-function …

Mar 1, 2020 · Our findings highlight a positive transcriptional-translational feedback regulation of the PRNP gene by PrP through dynamic unwinding of GQs in its promoter. Taken together, …

Dec 2, 2016 · To determine the prevalence of the PRNP gene in patients with AD and FTD in China, we screened all exons of the PRNP gene in a cohort of 683 cases (606 AD and 77 …

Mar 30, 2025 · PRNP (Prion Protein (Kanno Blood Group)) is a Protein Coding gene. Diseases associated with PRNP include Fatal Familial Insomnia and Gerstmann-Straussler Disease. …

Oct 13, 2022 · The essential role of host PrP for prion propagation and pathogenesis is demonstrated by the fact that knockout mice lacking PrP expression (Prnp o/o mice) are …

Starting with a brief overview of prion theory, this article examines findings that PRNP associates directly with the antimicrobial peptide Aβ, that PRNP and Aβ are codeposited in disease brain, …

Oct 18, 2021 · We describe the clinical and neuropathological data of inherited early-onset prion disease caused by the rare PRNP homozygous mutation R136S. In vitro PrP Sc propagation …