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Funding This work was supported by grants from the National Natural Science Foundation of China (No.82271847)

Mice were bred and maintained at the Lockey preclinical research facility of the Weizmann Institute of Science

Ubiquitin-linked mutant huntingtin has been found in neuronal intranuclear inclusions and dystrophic neurites in the cortex and striatum of HD patients (Difiglia et al., 1997)