PROTEIN QUALITY CONTROL IN ANIMAL MODELS OF ALS
Embargo until
2020-08-01
Date
2016-08-16
Authors
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Publisher
Johns Hopkins University
Abstract
Efficient and effective protein quality control are crucial components of cellular homeostasis in the presence of misfolded and aggregated proteins. One method for identifying the mechanistic targets of such protein quality control is via appropriate mouse models. Through exogenous alterations via methods such as drug injection on genetically modified mice, various crucial components of the protein quality control system may be identified. Such modulation could be induced in an effort to increase proteasome efficiency in clearing protein aggregates and alleviate dangerous levels of protein cytotoxicity. Thus in this experiment, a drug targeting the protein quality control system was administered to SOD1G93A transgenic mice to observe the potential alleviation of ALS symptoms. Drug efficacy was gauged based off of a rotarod balance test, grip strength test, and weight measurements. The drug’s efficacy in vivo was the main objective of this research project. Additional research is required to understand the full effects of the drug and any potential confounding mechanisms.
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Keywords
als, protein quality control