BRAIN: Jia Yichang's team found for the first time evidence of misprocessed stress particles in ALS
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Last Update: 2020-05-30
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Source: Internet
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Author: User
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Previous studies have shown that many RNA binding proteins, including TDP-43, FUS, and TIA1, are components of stress particles whose dysfunction causes amyotrophic lateral sclerosis (ALS)However, it is not clear whether the mutated RNA binding protein disrupts the process of the body's stress particles in the pathogenesisrecently, the Jia Yichang team at Tsinghua University established a FUS ALS mutation, p.R521C, in mice that carried impaired motor power and loss of advanced motor neuronsin disease-prone neurons, stress can induce mutants FUS to mislocate on stress particles, and ubiquitinization is two markers of ALS disease pathologyin addition, stress increases the motor performance of mutant miceBy using dual photon imaging in TIA1-EGFP transductioned animals, the researchers recorded the formation of more intense TIA1-EGFP-positive particles in a stress-challenged mutant cortex neuron, which took weeks to clearin addition, severe particle mistreated neurons died days after stresstherefore, the researchers believe that the mistreatment of stress particles is a causative factor in ALS, and the models they provide here provide a good basis for further study of disease mechanisms
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