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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Endoplasmic reticulum stress in neurodegenerative diseases

Table of Contents: “…Contents: Neurodegenerative disease -- Misfolded aggregates deposited in the brain -- Research strategy -- Prion diseases -- Two conformations of the prion protein -- Experimental scrapie model in mice -- Upregulation of ER chaperones -- ER-stress and UPR in human CJD -- PrP interaction with ER chaperones in brain -- PrPSc induction of ER-stress and neuronal death -- Calcineurin properties -- A novel treatment for prion diseases.…”
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Roles for Hsp40 molecular chaperones in protein misfolding disease

Table of Contents: “…Contents: Protein aggregation in conformational disease -- The Hsp40 system -- The Hsp70 system -- Small oligomers, prion, yeast, Huntington's disease and protein triage -- Mechanisms for proteotoxicity -- Mechanisms for amyloid disease.…”
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Protein degradation and defense against neurodegenerative disease part 2 of 2 /

Table of Contents: “…Contents: The 26S proteasome -- The 20S proteasome -- Proteasome inhibitors -- PS-341 -- Proteasome inhibitors in multiple myeloma -- Structures of the archaeal PAN ATPase-20S complex and the eukaryotic 26S proteasome -- Multiple ATP-dependent steps in proteasome function -- The gating mechanism of the proteasome -- Prions inhibit proteasome function -- Binding of ubiquitin conjugates activate the 26S proteasome by stimulating 20S gate-opening and ATP hydrolysis -- Ubiquitin conjugates activate their own degradation by the 26S Proteasome -- The inhibitor of proteasome-associated deubiquitinating enzyme Usp14, IU1, enhances degradation of tau.…”
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