Mutations in TAR DNA-binding protein 43 (TDP43) are associated with amyotrophic lateral sclerosis; however, the precise mechanisms of TDP43-mediated pathogenesis are unclear. The authors show that the specific expression of mutant TDP43 in spinal astrocytes is sufficient to cause progressive motor ...
PrpTDP-43A315T-UeGFP mice express a mutation of the human TDP43 gene and develop progressive motor neuron degeneration, leading to gait abnormalities, muscle weakness, and eventually death, mimicking key features of ALS. TDP43-associated pathology is found in approximately 97% of people with ALS...
www.nature.com/scientificreports OPEN Structural analysis of disease- related TDP-43 D169G mutation: linking enhanced stability and received: 02 September 2015 caspase cleavage efficiency toaccepted:25January2016 protein accumulationPublished:17February2016 Chien-Hao Chiang1,2, Cédric Grauffel3, ...
(b) (top) Schematic of TDP-43 protein demonstrating location of M337V point mutation; abbreviations as in a. (bottom) Sholl analysis for indicated conditions (Control, N = 60, TDP-43 overexpression, N = 60, TDP-43 M337V overexpression, N = 50). Two-Way ANOVA with Tukey...
【6】A. Patelet al., A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation.Cell162, 1066-1077 (2015). 【7】Y. Lin, D. S. Protter, M. K. Rosen, R. Parker, Formation and Maturation of Phase-Separated Liquid Droplets by RNA-Binding Proteins.Mol Ce...
Activation of ER stress and autophagy induced by TDP-43 A315T as pathogenic mechanism and the corresponding histological changes in skin as potential biomarker for ALS with the mutation[ J]. Int J Biol Sci, 2015, 11(10): 1140-1149. 36. Hu W, Liu X, Wang S, et al. SecinH3 ...
1 reagents referenced in Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis.
RNAstabilityandtransport.InaffectedneuronsoftransgenicrodentmodelswithTDP-43mutationoroverexpression,nuclearandcytoplasmicTDP一43aggregateswithubiquitinationorphosphorylation,andcellcyclesalsoalterate.HereresearchprogressontransgenicrodentmodelsmadebyTDP-43mutationoroverexpressionisreviewedtoexploremolecularmechanisms,pathological...
Here research progress on transgenic rodent models made by TDP-43 mutation or overexpression is reviewed to explore molecular mechanisms, pathological changes and new therapies for neurodegenerative disease. Key words: TDP-43; neurodegenerative disease; ALS; animal model收稿日期:2013-10-14; 修回日期:...
【6】A. Patelet al., A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated byDisease Mutation.Cell162, 1066-1077 (2015). 【7】Y. Lin, D. S. Protter, M. K. Rosen, R. Parker, Formation and ...