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 INTRODUCTION Alzheimer's disease (AD) features amyloid beta (Aβ) plaques and tau tangles, yet how their coexistence reshapes brain transcriptomic programs remains unclear. METHODS We performed high‐quality, sex‐balanced single‐nucleus RNA sequencing of 5XFAD (Aβ), PS19 (tau), and combined 5XFAD;PS19 mice.
Jung Hyun Park +9 more
wiley +1 more source
Single‐cell atlas of neuroglial dynamics in SNCA‐A53T Parkinson's disease mouse model
We performed single‐cell RNA sequencing of midbrain and striatal tissues from SNCA‐A53T Parkinson's disease (PD) mice, revealing glia‐enriched PD‐risk gene signatures and disease‐specific subpopulations. Transcriptional dysregulation of key TFs (e.g., Rorb, Foxc1) and enhanced neuroinflammatory signaling (SEMA, CCL, MIF) were identified.
Binqing Qin +7 more
wiley +1 more source
Comparative genomic organization of the human and bovine PRNP locus [PDF]
We sequenced a 208-kb BAC clone spanning the bovine prion protein (PRNP) locus, and compared the genomic structure with that of human. As a result, we determined the precise breakpoint between the two syntenic genomes, located on the 5′ UTR of the PRNP ...
Mi-Na Song +25 more
core +1 more source
Transgenic A53T Mice Have Astrocytic α‐Synuclein Aggregates in Dopamine and Striatal Regions
To identify whether astrocytes accumulate α‐synuclein aggregates before or after neurons, astrocytes from the midbrain and striatum of M83 A53T transgenic mice were compared to wild‐type. The substantia nigra pars compacta had more astrocytes than the ventral tegmental area or striatum, and small α‐synuclein aggregates were observed even within wild ...
Cormac Peat +8 more
wiley +1 more source
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
FMRP‐Mediated Proteasome Regulation: A Novel Mechanism in ALS Pathology
Schematic model of TDP‐43/TNKS‐mediated proteasome regulation in WT, FMRP‐depleted, and TDP‐43A315T‐Tg ALS neurons. In WT neurons, cytoplasmic TDP‐43 partially sequesters TNKS, maintaining balanced PI31 ribosylation and proteasome activity. FMRP depletion promotes nuclear translocation of TDP‐43, enhances TNKS/PI31 interaction, and increases axonal ...
Pritha Majumder +5 more
wiley +1 more source
TDP‐43 Aggregation: The Healthy‐Toxic Balance of the Prion‐Like Domain
TDP‐43 function relies on a delicate balance between reversible phase‐separated states and irreversible aggregation. Under physiological conditions, TDP‐43 forms dynamic droplets and oligomers that support normal cellular functions. In pathological contexts, this balance shifts toward aberrant aggregation, leading to toxic species.
Luca Zangrando +2 more
wiley +1 more source
A Practical Framework for GT‐Seq Panel Optimization
ABSTRACT Genotyping‐in‐thousands by sequencing (GT‐seq) panels are powerful tools in ecological, evolutionary and conservation genomics, yet the optimization process critical for robust and reproducible genotyping remains poorly formalized. Here, we present an iterative workflow for GT‐seq panel optimization that emphasizes systematic refinement ...
Chandika RG +8 more
wiley +1 more source
Prion Protein Gene (PRNP) variation in German and Danish cervids [PDF]
The structure of cellular prion proteins encoded by the Prion Protein gene (PRNP) impactssusceptibility to transmissible spongiform encephalopathies, including Chronic Wasting Disease (CWD) in deer.
Dolores Gavier-Widén +23 more
core +1 more source

