Results 21 to 30 of about 8,300 (285)
Hepatology, EarlyView., 2022 Hepatic endothelial Alk1 signaling protects from development of vascular malformations while maintaining organ‐specific endothelial differentiation and angiocrine portmanteau of the names Wingless and Int‐1 signaling. Abstract Background and Aims In hereditary hemorrhagic telangiectasia (HHT), severe liver vascular malformations are associated with ...
Christian David Schmid +20 more
wiley +1 more source
Chronic Wasting Disease Prion Strain Emergence and Host Range Expansion
Emerging Infectious Diseases, 2017 Human and mouse prion proteins share a structural motif that regulates resistance to common chronic wasting disease (CWD) prion strains. Successful transmission of an emergent strain of CWD prion, H95+, into mice resulted in infection. Thus, emergent CWD
Allen Herbst +4 more
doaj +1 more source
Assessing Prion Infectivity of Human Urine in Sporadic Creutzfeldt-Jakob Disease
Emerging Infectious Diseases, 2012 Prion diseases are neurodegenerative conditions associated with a misfolded and infectious protein, scrapie prion protein (PrPSc). PrPSc propagate prion diseases within and between species and thus pose risks to public health.
Silvio Notari +12 more
doaj +1 more source
Curing of the [URE3] prion by Btn2p, a Batten disease‐related protein [PDF]
The EMBO Journal, 2008 [URE3] is a prion (infectious protein), a self-propagating amyloid form of Ure2p, a regulator of yeast nitrogen catabolism. We find that overproduction of Btn2p, or its homologue Ypr158 (Cur1p), cures [URE3]. Btn2p is reported to be associated with late endosomes and to affect sorting of several proteins.
Dmitry S, Kryndushkin +2 more
openaire +2 more sources
Transmission Characteristics of Variably Protease-Sensitive Prionopathy
Emerging Infectious Diseases, 2014 Variably protease-sensitive prionopathy (VPSPr), a recently identified and seemingly sporadic human prion disease, is distinct from Creutzfeldt-Jakob disease (CJD) but shares features of Gerstmann-Sträussler-Scheinker disease (GSS).
Silvio Notari +11 more
doaj +1 more source
Defining the Protein Seeds of Neurodegeneration using Real-Time Quaking-Induced Conversion Assays
Biomolecules, 2020 Neurodegenerative diseases are characterized by the accumulation of disease-related misfolded proteins. It is now widely understood that the characteristic self-amplifying (i.e., seeding) capacity once only attributed to the prions of transmissible ...
Matteo Manca, Allison Kraus
doaj +1 more source
Can loss-of-function prion-related diseases exist? [PDF]
Molecular Psychiatry, 1998 Discovery of mutations of the PrnP gene without typical plaque formation and the characterization of two prion receptors led us to postulate a new class of prion-related disease: ‘loss of function’.
H B Samaia, R R Brentani
openaire +1 more source
Emerging Infectious Diseases, 2021 Chronic wasting disease (CWD) is a fatal prion disease of cervids. We examined host range of CWD by oronasally inoculating Suffolk sheep with brain homogenate from a CWD-positive white-tailed deer.
Eric D. Cassmann +2 more
doaj +1 more source
Insufficient hydrogen‐bond desolvation and prion‐related disease [PDF]
European Journal of Biochemistry, 2002 A structuring and eventual exclusion of water surrounding backbone hydrogen bonds takes place during protein folding as hydrophobic residues cluster around such bonds. Taken as an average over all hydrogen bonds, the extent of desolvation is nearly a constant of motion, as revealed by re‐examination of the longest all‐atom trajectory with explicit ...
openaire +2 more sources
Emerging Infectious Diseases, 2017 In the United-Kingdom, ≈1 of 2,000 persons could be infected with variant Creutzfeldt-Jakob disease (vCJD). Therefore, risk of transmission of vCJD by medical procedures remains a major concern for public health authorities.
Jean Y. Douet +11 more
doaj +1 more source