Results 51 to 60 of about 1,549,882 (213)
Challenges and Advances in Antemortem Diagnosis of Human Transmissible Spongiform Encephalopathies
Frontiers in Bioengineering and Biotechnology, 2020 Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, arise from the structural conversion of the monomeric, cellular prion protein (PrPC) into its multimeric scrapie form (PrPSc).
Lucas M. Ascari +4 more
doaj +1 more source
Combating ageing beyond the cell: Emerging roles of extracellular proteostasis
The FEBS Journal, Volume 293, Issue 18, Page 5558-5584, September 2026.Ageing challenges the body's ability to maintain a stable and functional proteome, leading to protein damage and aggregation both inside and outside cells. This review focuses on the less well understood mechanisms of extracellular protein quality control and how they become disrupted in ageing, particularly in neurodegenerative diseases.
Siddharth R. Venkatesh +7 more
wiley +1 more source
PrP(Sc)-specific antibodies with the ability to immunodetect prion oligomers.
PLoS ONE, 2011 The development of antibodies with binding capacity towards soluble oligomeric forms of PrPSc recognised in the aggregation process in early stage of the disease would be of paramount importance in diagnosing prion diseases before extensive ...
Mourad Tayebi +6 more
doaj +1 more source
Human Prion Protein (PrP) 219K Is Converted to PrPSc but Shows Heterozygous Inhibition in Variant Creutzfeldt-Jakob Disease Infection [PDF]
Journal of Biological Chemistry, 2009 Prion protein gene (PRNP) E219K is a human polymorphism commonly occurring in Asian populations but is rarely found in patients with sporadic Creutzfeldt-Jakob disease (CJD). Thus the polymorphism E219K has been considered protective against sporadic CJD. The corresponding mouse prion protein (PrP) polymorphism variant (mouse PrP 218K) is not converted
Masaki, Hizume +6 more
openaire +2 more sources
Advanced Science, Volume 13, Issue 45, 13 August 2026.The aptamer WHY‐3E identifies PrPC as a CRC driver. Stabilized by USP18, endocytosed PrPC forms a LYN/STAT3 complex, upregulating MSN transcription to promote metastasis. Crucially, WHY‐3E sensitively detects PrPC‐positive circulating exosomes, establishing a robust theoretical foundation for non‐invasive clinical diagnostics.
Chunlin Wang +23 more
wiley +1 more source
Phosphatidylinositol-glycan-phospholipase D is involved in neurodegeneration in prion disease. [PDF]
PLoS ONE, 2015 PrPSc is formed from a normal glycosylphosphatidylinositol (GPI)-anchored prion protein (PrPC) by a posttranslational modification. Most GPI-anchored proteins have been shown to be cleaved by GPI phospholipases.
Jae-Kwang Jin +8 more
doaj +1 more source
Distinct Structures of Scrapie Prion Protein (PrPSc)-seeded Versus Spontaneous Recombinant Prion Protein Fibrils Revealed by Hydrogen/Deuterium Exchange [PDF]
Journal of Biological Chemistry, 2009 The detailed structures of prion disease-associated, partially protease-resistant forms of prion protein (e.g. PrP(Sc)) are largely unknown. PrP(Sc) appears to propagate itself by autocatalyzing the conformational conversion and oligomerization of normal prion protein (PrP(C)). One manifestation of PrP(Sc) templating activity is its ability, in protein
Vytautas, Smirnovas +5 more
openaire +2 more sources
Rapid generation of prion disease models using AAV‐delivered PrP variants in knockout mice
Brain 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
MRI characteristics of sporadic CJD with valine homozygosity at codon 129 of the prion protein gene and PrPSc type 2 in Japan [PDF]
Journal of Neurology, Neurosurgery & Psychiatry, 2004 Two Japanese sporadic Creutzfeld-Jakob disease (sCJD) patients with valine homozygosity at codon 129 of the prion protein gene and protease-resistant prion protein (PrP(Sc)) type 2 (VV2) are described. In contrast with Western countries, this type of sCJD is very rare in Japan.
R, Fukushima +5 more
openaire +2 more sources
Brain 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