Prion Diseases and Protein Misfolding

Glial activation in prion diseases is strictly nonautonomous and requires neuronal PrPSc

The paper shows that glial activation in prion diseases is nonautonomous, requiring neuronal PrPSc to drive neural dysfunction.

Identification of potential key genes and pathway linked with sporadic Creutzfeldt-Jakob disease based on integrated bioinformatics analyses

The paper identifies key genes and pathways associated with sporadic Creutzfeldt-Jakob disease using bioinformatics analyses of gene expression data.

The role of macrophage scavenger receptor 1 (Msr1) in prion pathogenesis

This study investigates the role of Msr1 in prion clearance and pathogenesis, finding that Msr1 deficiency does not affect prion disease progression or neuroinflammation.

Endosomal Sorting Drives the Formation of Mutant Prion Endoggresomes

The paper identifies endosomal sorting pathways that drive the formation of mutant prion endoggresomes and shows that inhibiting this process can reduce axonal dysfunction.

Prions induce minor genome-wide translational changes in neurons compared to glia

This study uses ribosome profiling to show that prion disease causes minor translational changes in neurons but significant changes in glia, overlapping with other neurodegenerative diseases.

Protective anti-prion antibodies in human immunoglobulin repertoires

The paper identifies protective anti-prion antibodies in human immunoglobulin repertoires and finds they are present in healthy individuals without associated pathologies.

Prion protein lowering is a disease-modifying therapy across prion strains, disease stages, and endpoints

This study shows that lowering prion protein expression with ASOs is effective in treating prion disease across different strains, treatment times, and endpoints.

Prion infection, transmission and cytopathology modelled in a low-biohazard human cell line

The paper describes a human cell line, hovS, that can be infected with sheep-derived prions and exhibits prion-related cytopathology, offering a safer alternative to human prion studies.

Genome-wide transcriptomics identifies an early preclinical signature of prion infection

The paper identifies early molecular changes in prion-infected mice, suggesting glial pathophysiology as a key driver and potential targets for intervention.

A unifying model for the propagation of prion proteins in yeast brings insight into the prion

The paper introduces a mathematical model to study prion propagation in yeast, revealing a bi-stable switch mechanism and new insights into prion curing and aggregate replication.

An alternative model to prion fragmentation based on the detailed balance between PrPSc and suPrP

The paper compares two prion growth models, showing that the detailed balance between PrPSc and suPrP can replace the induced-fragmentation model.

Natural and Pathogenic Protein Sequence Variation Affecting Prion-Like Domains Within and Across Human Proteomes

The paper examines how sequence variation in prion-like domains (PrLDs) affects aggregation propensity in human proteins, identifying mutations linked to increased aggregation and disease.

Age structuring and spatial heterogeneity in prion protein gene (PRNP) polymorphism in white-tailed deer

The paper quantifies the geographic distribution of PRNP variants in white-tailed deer to assess CWD susceptibility and management implications.

Molecular dynamics simulation of local structural models of PrPSc reveals how codon 129 polymorphism affects propagation of PrPSc.

The paper uses molecular dynamics simulations to show how codon 129 polymorphism affects PrPSc structure and propagation by comparing V129 and M129 models.

Complementation between pathological prion protein subassemblies to cross existing species barriers

The paper shows that structurally diverse PrPSc assemblies complement each other to cross species barriers, supporting the quasi-species concept in prions.

Possible origin of the scrapie genome in small endogenous RNAs; studies on eight candidate species in 263K scrapie-infected hamster brain

The paper investigates the role of small endogenous RNAs in scrapie-infected hamster brains but finds no scrapie-specific variants in eight candidate RNA species.

Loss of region-specific glial homeostatic signature in prion diseases

The paper examines how prion diseases alter region-specific glial homeostatic signatures, finding that these signatures are replaced by a region-independent neuroinflammation signature during disease progression.

The amino acid residue in position 163 of canine PrPC is critical to the exceptional resistance of dogs to prion infections: evidence from transgenic mouse models

The paper shows that a specific amino acid at position 163 in canine PrPC confers resistance to prion infection, confirmed through transgenic mouse models.

Domain-specific quantification of prion protein in cerebrospinal fluid by targeted mass spectrometry

The paper develops a targeted mass spectrometry method to quantify prion protein in CSF, confirming that reduced PrP levels in prion disease are due to the disease process, not ELISA artifacts.

Transmission of prion infectivity from blood by peptoid-conjugated beads

This study demonstrates that peptoid-conjugated beads can capture and transmit prion infectivity from blood, validating a sensitive assay for prion detection.

Age of onset in genetic prion disease and the design of preventive clinical trials

This paper quantifies the challenges of designing preventive clinical trials for genetic prion disease due to high variability in age of onset, suggesting the need for biomarker-based trials.

Yeast Sup35 prion structure: two types, four parts, many variants

The paper maps amyloid cores of 26 [PSI+] prion isolates and identifies two distinct structural classes based on digestion patterns and phenotypic effects.

In silico evidence of unique behaviors of methionine in an in-register parallel beta-sheet amyloid, suggestive of possible contribution to strain diversity of amyloids.

The paper uses molecular dynamics simulations to show that methionine uniquely stabilizes a beta-arch structure in an in-register parallel beta-sheet amyloid, suggesting its role in prion strain diversity.

Prion protein quantification in cerebrospinal fluid as a tool for prion disease drug development

This study evaluates ELISA-based quantification of PrP in CSF as a biomarker for PrP-reducing therapeutics, showing its reliability and CNS origin.

SARM1 deficiency, which prevents Wallerian degeneration, upregulates XAF1 and accelerates prion disease

This study shows that SARM1 deficiency accelerates prion disease progression by upregulating XAF1, leading to increased neuronal apoptosis.

Mechanisms of strain diversity of disease-associated in-register parallel β-sheet amyloids and implications about prion strains.

The paper reviews advances in understanding the structures of Tau and Syn amyloids and discusses their implications for prion strain diversity mechanisms.

Unaltered prion disease in mice lacking developmental endothelial locus-1 (Del 1)

This study investigates the role of Del-1 in prion disease progression and finds that its absence does not affect prion pathogenesis, clearance, or neuroinflammation in mice.

A bispecific immunotweezer prevents soluble PrP oligomers and abolishes prion toxicity

This paper introduces a bispecific antibody that targets both the globular domain and flexible tail of PrP, preventing prion toxicity and oligomer formation.

Lymphocyte activation gene 3 (Lag3) expression is increased in prion infections but does not modify disease progression

This study finds that Lag3 expression increases in prion infections but does not affect disease progression in knockout mice.

Structure analysis of human Prion protein involved in Sporadic Fatal Insomnia

This study analyzes the structure of the native human Prion Protein and compares it with the toxic form causing Sporadic Fatal Insomnia, finding increased flexibility in the mutant form.