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• W2897417379 abstract "Neuronal and non-neuronal cells express the huntingtin (HTT) protein, yet neurodegeneration in Huntington’s disease (HD) is largely selective, affecting most prominently striatal medium spiny neurons and cortical pyramidal neurons. Selective toxicity of full-length human mutant HTT (fl-mHTT) may be due in part to its expression in non-neuronal cells. While studies suggest neuronal–glial interactions are important in HD and fl-mHTT is expressed in astrocytes, it has not been determined whether the expression of fl-mHTT in astrocytes is necessary for HD pathogenesis. To directly assess the necessity of fl-mHTT in astrocytes for HD pathogenesis, we used a mouse genetic approach and bred the conditional mHTT-expressing BACHD mouse model with GFAP-CreERT2 mice. We show that GFAP-CreERT2 expression in these mice is highly selective for astrocytes, and we are able to significantly reduce the expression of fl-mHTT protein in the striatum and cortex of BACHD/GFAP-CreERT2-tam mice. We performed behavioral, electrophysiological and neuropathological analyses of BACHD and BACHD/GFAP-CreERT2-tam mice. Behavioral analyses of BACHD/GFAP-CreERT2-tam mice demonstrate significant improvements in motor and psychiatric-like phenotypes. We observe improvements in neuropathological and electrophysiological phenotypes in BACHD/GFAP-CreERT2-tam mice compared to BACHD mice. We observed a restoration of the normal level αB-crystallin in the striatum of the BACHD/GFAP-CreERT2 mice, indicating a cell autonomous effect of mHTT on its expression. Taken together, this work indicates that astrocytes are important contributors to the progression of the behavioral and neuropathological phenotypes observed in HD." @default.
• W2897417379 created "2018-10-26" @default.
• W2897417379 creator A5004961699 @default.
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• W2897417379 date "2018-10-12" @default.
• W2897417379 modified "2023-10-14" @default.
• W2897417379 title "Mutant huntingtin reduction in astrocytes slows disease progression in the bachd conditional huntington’s disease mouse model" @default.
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• W2897417379 doi "https://doi.org/10.1093/hmg/ddy363" @default.
• W2897417379 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6337698" @default.
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• W2897417379 hasPublicationYear "2018" @default.
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