Mouse-hamster chimeric prion protein (PrP) devoid of N-terminal residues 23-88 restores susceptibility to 22L prions, but not to RML prions in PrP-knockout mice. [PDF]
Prion infection induces conformational conversion of the normal prion protein PrPC, into the pathogenic isoform PrPSc, in prion diseases. It has been shown that PrP-knockout (Prnp0/0) mice transgenically reconstituted with a mouse-hamster chimeric PrP ...
Keiji Uchiyama +9 more
doaj +1 more source
ABSTRACT Oncogenic condensates act as biophysical sanctuaries that stabilize malignant survival programs. However, a universal regulator capable of orchestrating the integrated biophysical axes governing cellular phase behavior has remained elusive.
Doris Loh +3 more
wiley +1 more source
HaloTag Fusion Enables Dynamic Analysis of Prion Protein Biosynthesis, Turnover, and Misfolding
Prion protein (PrP) misfolding underlies fatal neurodegenerative diseases. We developed a HaloTag‐based PrP fusion enabling spatiotemporal labeling of distinct PrP populations in living cells. This system recapitulates native PrP biology, reveals early misfolding events in disease‐associated mutants, and allows mechanistic interrogation of PrP‐lowering
Antonio Masone +2 more
wiley +1 more source
Poliovirus type 1 infection of murine PRNP-knockout neuronal cells
Transfection of the prion protein gene (Prnp) into prion-deficient mouse cells was shown to reduce the replication of coxsackievirus B3, an enterovirus. Because mice can be susceptible to poliovirus infection by parenteral routes, the authors tested the ...
NISHIMURA T +4 more
core +1 more source
Mouse neuronal cells expressing exogenous bovinePRNPand simultaneous downregulation of endogenous mousePRNPusing siRNAs [PDF]
Prion diseases, which are called transmissible spongiform encephalopathies (TSEs), comprise a group of fatal infectious neurodegenerative disorders. Investigation of prion strains and generation of species dependent TSE model are necessary to understand pathogenesis of the disease.
Sang-Gyun, Kang +4 more
openaire +2 more sources
Mapping cross‐domain drivers of Alzheimer's disease risk through integrated network analysis
Abstract INTRODUCTION Alzheimer's disease (AD) is a complex neurodegenerative disorder with numerous known risk factors. Identification of which genetic factors are causal drivers is difficult due to the long disease prodrome in an inaccessible organ.
Gregory A Cary +10 more
wiley +1 more source
Mitochondrial NADK2‐dependent NADPH controls tau oligomer uptake in human neurons
Abstract INTRODUCTION Reduced brain energy metabolism, mitochondria dysfunction, and extracellular tau oligomer buildup characterize Alzheimer's disease (AD), but how these phenomena cooperatively promote neurodegeneration is poorly understood. We now report that tau oligomers (TauOs) pathologically coordinate mitochondrial metabolism with increased ...
Evelyn Pardo +10 more
wiley +1 more source
Molecular Characterization of the Rocky Mountain Elk (Cervus elaphus nelsoni) PRNP Putative Promoter [PDF]
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
Association analysis of
Background Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) of cervids including white-tailed (Odocoileus virginianus) and mule deer (Odocoileus hemionus), Rocky Mountain elk (Cervus elaphus nelsoni), and moose (Alces ...
Spraker Terry R +3 more
doaj +1 more source
Exploring and Targeting the Connection of Iron and Copper Homeostasis to Neurodegenerative Diseases
Iron and copper dyshomeostasis, along with their interactions with key intrinsically disordered proteins (e.g., Aβ, tau, α‐synuclein) have a strong implication in the onset and progression of neurodegenerative diseases, including Alzheimer's disease (AD), Parkinson's disease (PD), Prion diseases (PrDs), Huntington's disease (HD), Wilson's disease (WD),
Xin Liu +9 more
wiley +1 more source

