Search Results - "prion"

Pathogenic mechanisms in prion disease

Table of Contents: “…Contents: Neurodegenerative diseases and protein misfolding disorders -- Prion disease in mice to study mechanisms of neurodegeneration due to misfolded protein accumulation -- Early prion neurodegenerative change is reversible: access to key neurotoxic pathways -- Critical change is loss of synaptic proteins due to activation of PERK branch of the unfolded protein response -- Modifying this pathway genetically is neuroprotective -- Modifying this pathway pharmacologically is neuroprotective -- Relevance of this pathway in other neurodegenerative diseases with misolded proteins e.g. …”
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Expanding roles of RNA-binding proteins in neurodegenerative diseases

Table of Contents: “…Contents: Amyotrophic lateral sclerosis (ALS) -- Genetic causes of ALS -- Role of RNA-binding proteins and stress granules in ALS pathogenesis -- Emerging class of RNA-binding proteins with prion-like domains in neurodegenerative disease -- Mechanisms by which C9ORF72 mutations cause ALS.…”
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Neurodegenerative disease expression levels of normal sequence pathogenic proteins contribute to risk of disease /

Table of Contents: “…Contents: Protein pathology of disease -- Loci underlying autosomal dominant neurodegenerative disease -- Prion mutations causing Gerstmann Straussler syndrome and hereditary Creuzfeldt-Jakob disease -- Tau mutations cause autosomal dominant frontal temporal dementia -- Alpha-synuclein mutations cause autosomal dominant Parkinson's disease -- How genetic variability of these genes contributes to the risk of sporadic disease.…”
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