Uninterrupted CAG Repeat Drives Striatum-Selective Transcriptionopathy and Nuclear Pathogenesis in Human Huntingtin BAC Mice.
Neuron（2022）
关键词
Huntingtin,mutant huntingtin,Huntington’s disease,HD,BAC transgenic,CAG repeat,BAC-CAG,instability,polyglutamine,nuclear inclusions,aggregation,motor deficits,RNA-seq,transcriptional dysregulation,transcriptionopathy,striatum,BACHD,knockin,RAN translation,muscleblind,RNA foci
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