专题:Prion Diseases and Protein Misfolding

This cluster of papers focuses on the causes, molecular basis, detection, transmission, and potential treatments for prion diseases, including variant Creutzfeldt-Jakob disease. It explores the role of infectious prions, protein misfolding, and their impact on neurodegenerative diseases. The research also delves into the pathology of these diseases and their transmissibility through blood transfusion.
最新文献
The amyloid packing difference: a pairwise comparison metric for amyloid structures

article Full Text OpenAlex

Breaking β-sheets in FUS prion-like domain preserves phase separation and function but prevents aggregation and toxicity

article Full Text OpenAlex

Membrane-anchored PrPSc is the trigger for prion synaptotoxicity

article Full Text OpenAlex

Presymptomatic pharmacological inhibition of mGluR5 improves survival in a mouse model of prion diseases

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The multistep pathogenic hypothesis of amyotrophic lateral sclerosis is incompatible with the epidemiological data

article Full Text OpenAlex

Advances in biomarkers for diagnosis and prognosis of prion diseases

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Heterogeneous Dynamics of the Fuzzy Coat of Full-Length Phospho-Mimetic Tau Fibrils

article Full Text OpenAlex

Defining RNA oligonucleotides that reverse deleterious phase transitions of RNA-binding proteins with prion-like domains

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Alpha-synuclein seeding activity in postmortem tissues from patients with diffuse and isolated Lewy bodies

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Spectrum of dominant Charcot-Marie-Tooth disease due to SLC12A6 variants

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近5年高被引文献
Cryo-EM structures of amyloid-β 42 filaments from human brains

article Full Text OpenAlex 422 FWCI49.8317

Emerging insights into the complex genetics and pathophysiology of amyotrophic lateral sclerosis

review Full Text OpenAlex 364 FWCI43.1107

In vivo staging of regional amyloid deposition

article Full Text OpenAlex 308 FWCI13.2234

Amyloid nomenclature 2022: update, novel proteins, and recommendations by the International Society of Amyloidosis (ISA) Nomenclature Committee

article Full Text OpenAlex 301 FWCI25.6165

Anti-Amyloid Monoclonal Antibodies for the Treatment of Alzheimer’s Disease

review Full Text OpenAlex 281 FWCI50.0957

Molecular pathology of neurodegenerative diseases by cryo-EM of amyloids

review Full Text OpenAlex 231 FWCI41.2694

Condensates formed by prion-like low-complexity domains have small-world network structures and interfaces defined by expanded conformations

article Full Text OpenAlex 225 FWCI18.3083

Structure-based discovery of small molecules that disaggregate Alzheimer’s disease tissue derived tau fibrils in vitro

article Full Text OpenAlex 176 FWCI19.4808

Disease-specific tau filaments assemble via polymorphic intermediates

article Full Text OpenAlex 170 FWCI26.6064

The interface of condensates of the hnRNPA1 low-complexity domain promotes formation of amyloid fibrils

article Full Text OpenAlex 165 FWCI25.6673