Background: Aberrant activation of autophagy (proteinopathy) leads to neurodegeneration in various neurological disorders. Results: Compensatory basal autophagy is activated, and genotoxins deregulate autophagy in the ubiquitin pathway-compromised ataxia telangiectasia (A-T) cells. Conclusion: Deregulation of autophagy is due to the elevated expression of ISG15 in A-T cells. Significance: Results highlight a causal contribution of a novel "ISG15 proteinopathy" in A-T neurodegeneration.
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