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Structural and functional features of medium spiny neurons in the BACHDΔN17 mouse model of Huntington’s disease

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DataONE2024-07-18 更新2025-04-26 收录
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In the BACHD mouse model of Huntington’s disease (HD), deletion of the N17 domain of the Huntingtin gene (BACHDΔN17, Q97) has been reported to lead to nuclear accumulation of mHTT and exacerbation of motor deficits, neuroinflammation and striatal atrophy (Gu et al., 2015). Here we characterized the effect of N17 deletion on dorsolateral striatal medium spiny neurons (MSNs) in BACHDΔN17 (Q97) and BACWTΔN17 (Q31) mice by comparing them to MSNs in wildtype (WT) mice. Mice were characterized on a series of motor tasks and subsequently whole cell patch clamp recordings with simultaneous biocytin filling of MSNs in in vitro striatal slices from these mice were used to comprehensively assess their physiological and morphological features. Key findings include that: Q97 mice exhibit impaired gait and righting reflexes but normal tail suspension reflexes and normal coats while Q31 mice do not differ from WT; intrinsic membrane and action potential properties are altered -but differentially so- i..., Behavior Data Videos:    Mice were weighed and assessed for quality of their coat prior to being assessed on three different tests of motor function including: tail suspension, self-righting, and gait assessment [49]. Coat quality was rated on a scale of 0 (normal, shiny)- 4 (matted, unkempt, yellowing). During testing, mice were videotaped and subsequently videos were analyzed by three researchers blinded to gender and genotype. For tail suspension, mice were suspended by their tails, one third of the length away from the base of the tail. Mice were suspended and videotaped for 15 seconds. The tail suspension task was scored on a scale of 0 (normal splaying of toes and legs)- 4 (curled toes, both legs close to body) For assessment of self-righting ability, mice were placed onto their backs and then released. This was repeated twice and videotaped for 20 seconds each time. Self-righting was scored as 0 (mouse rights self under 5 seconds)- 4 (mouse takes more than 5 seconds to right itse..., , # Data from: Structural and functional features of medium spiny neurons in the BACHDΔN17 mouse model of Huntington’s disease ## Description of the data and file structure  Our raw data for motor behavior (video files: each_individual_subject.MOV); rubric for scoring of the motor behavior (behavior rubric for scoring.xlsx); DN17 Behavior Data for Figure 1.xlsx  Note that in the behavior by phenotype datasheet values for the different groups are color-coded as follows: black- WT; blue- Q31; red- Q97.  Our raw data for electrophysiological assessments including Synaptic Events raw data (.ABF and .IBW files generated by MiniAnalysis from .DAT files for excitatory -EPSC- and inhibitory -IPSC- synaptic currents); raw basic membrane property data (Electrophysiology raw data files; .DAT files generated by PatchMaster for ALL physiological data recorded from each cell for each subject); and related excel files with numeric data (.xlsx)  Our raw data analyses spreadsheets for morphological var...
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2024-07-19
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