Fig. 1. Neurobehavioral testing in YG8R mice transplanted with wild-type mouse HSPCs. Neurobehavioral testing in YG8R mice transplanted with wild-type.

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Fig. 1. FMRP deficiency leads to increased NSC activation in the adult dentate gyrus. FMRP deficiency leads to increased NSC activation in the adult dentate.

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Molecular Therapy - Methods & Clinical Development

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Molecular Therapy - Methods & Clinical Development

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Volume 90, Issue 3, Pages (May 2016)

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Fig. 1. Neurobehavioral testing in YG8R mice transplanted with wild-type mouse HSPCs. Neurobehavioral testing in YG8R mice transplanted with wild-type mouse HSPCs. (A) Neurobehavioral testing (open field, rotarod, gait, and grip strength) was performed for wild-type (WT) mice (n = 16), nontransplanted YG8R control mice (YG8R; n = 4), YG8R mice transplanted with YG8R mfxn−/− hFXN+ HSPCs (YG8R/YG8R HSPCs; n = 5), and YG8R mice transplanted with wild-type HSPCs (YG8R/WT HSPCs; n = 13) at both 5 and 9 months of age. Locomotor activity was tested using an open-field test, coordination was tested using a rotarod test, gait was tested using an automated gait analysis system, and muscle strength was tested using a forelimb grip strength test. Data are means ± SEM. *P < 0.05, **P < 0.005 and ***P < 0.0005. For statistical comparison of three experimental groups, a mixed analysis of variance (ANOVA) with age of testing as a within-subjects variable was used, followed by independent sample t test. (B) Nissl-stained sections of the lumbar DRGs (L5) from representative 9-month-old wild-type mice (n = 15), YG8R control mice (n = 4), YG8R/YG8R HSPCs (n = 4), and YG8R/WT HSPCs (n = 11). Large vacuoles are shown by red arrows. Scale bars, 100 μm. Graph depicts total vacuolar area per DRG area (right). Data are means ± SEM. **P < 0.005 and ***P < 0.0005 (Mann-Whitney nonparametric test corrected for multiple testing by the Bonferroni correction). (C) Representative confocal images from a WT GFP+ HSPC-transplanted YG8R mouse 7 months after transplantation, stained with anti-GFP (green) and anti-NeuN (red) antibodies. Left: Image of a DRG. Scale bar, 100 μm (top). Magnified image with scale bar, 20 μm (below). Right: Images of cervical, thoracic, and lumbar spinal cord. Scale bars, 250 μm. (D) Confocal images of the DRG and spinal cord from a YG8R mouse transplanted with GFP+ HSPCs show engrafted cells (GFP; green) with neurons (NeuN; blue) and the Iba1 microglial marker (red). Scale bars, 30 μm. Celine J. Rocca et al., Sci Transl Med 2017;9:eaaj2347 Published by AAAS