SOD1 aggregation in ALS mice shows simplistic test tube behavior
作者： Lisa Lang , Per Zetterström , Thomas Brännström , Stefan L. Marklund , Jens Danielsson
关键词: Mutation 、 Unfolded protein response 、 In vitro 、 Genetically modified mouse 、 SOD1 、 Superoxide dismutase 、 Protein aggregation 、 In vivo 、 Neuroscience 、 Biology
摘要: A longstanding challenge in studies of neurodegenerative disease has been that the pathologic protein aggregates live tissue are not amenable to structural and kinetic analysis by conventional methods. The situation is put focus current progress demarcating aggregation vitro, exposing new mechanistic details now calling for quantitative vivo comparison. In this study, we bridge gap presenting a direct comparison kinetics ALS-associated superoxide dismutase 1 (SOD1) vitro transgenic mice. results based on sampling antibody assays show SOD1 fibrillation mirror with remarkable accuracy spinal cord aggregate buildup progression This similarity between data suggests that, despite complexity tissue, follows robust simplistic rules, providing insights into ALS pathology organism-level manifestation phenomena general.
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