24 research outputs found

    Precise Measurement of B Meson Lifetimes with Hadronic Decay Final States

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    Death of lumbar motor neurons is not a disease feature of <i>Smn</i><sup><i>2B/-</i></sup> SMA mice.

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    (A) ChAT immunostaining of motor neurons in the L1 spinal segment from control (Smn 2B/+) and SMA (Smn2B/-) mice at P16. Scale bars: 50μm. (B) Total number of L1 motor neurons from the same groups as in (A). The box-and-whiskers graph shows the individual values, median, interquartile range, minimum and maximum from 5 mice per group. Statistics were performed with two-tailed unpaired Student’s t-test. ns = not significant. (C) ChAT immunostaining of motor neurons in the L2 spinal segment from control (Smn 2B/+) and SMA (Smn2B/-) mice at P16. Scale bars: 50μm. (D) Total number of L2 motor neurons from the same groups as in (C). The box-and-whiskers graph shows the individual values, median, interquartile range, minimum and maximum from 5 mice per group. Statistics were performed with two-tailed unpaired Student’s t-test. ns = not significant. (E) ChAT immunostaining of motor neurons in the L5 spinal segment from control (Smn 2B/+) and SMA (Smn2B/-) mice at P16. Scale bars: 50μm. (F and G) Total number of L5 LMC (F) and L5 MMC (G) motor neurons from the same groups as in (E). The box-and-whiskers graph shows the individual values, median, interquartile range, minimum and maximum from Smn2B/+ (n = 4) and Smn2B/- (n = 5) mice. Statistics were performed with two-tailed unpaired Student’s t-test. ns = not significant.</p

    Analysis of Smn expression from the <i>Smn</i><sup><i>2B</i></sup> allele in the mouse spinal cord.

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    (A) Western blot analysis of Smn levels in the spinal cord from Smn+/+ (wild type) and Smn2B/2B mice at P16. (B) Western blot analysis of Smn levels in the spinal cord from Smn2B/+ an Smn2B/- mice at P16. Two-fold serial dilutions of equal amounts of extracts are shown. Tubulin was probed as a loading control. (TIF)</p

    <i>Smn</i><sup><i>2B/-</i></sup> SMA mice do not display gender-specific phenotypic differences in behavior.

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    (A) Body weight of male (n = 14) and female (n = 17) Smn2B/- SMA mice. Data represent mean and SEM. Statistics were performed with two-way ANOVA and Bonferroni’s multiple comparison test. Not Significant. (B) Kaplan-Meier survival curves from the same experimental groups as in (A). Statistics were performed with Log-rank (Mantel-Cox) test. Not Significant. (C) Righting time from the same experimental groups shown in (A). Data represent mean and SEM. Statistics were performed with two-way ANOVA and Bonferroni’s multiple comparison test. Not Significant. (D) Time to fall in the hindlimb suspension test from the same experimental groups shown in (A). Data represent mean and SEM. Statistics were performed with two-way ANOVA and Bonferroni’s multiple comparison test. Not Significant. (TIF)</p

    Smn deficiency induces p53 accumulation but not serine 18 phosphorylation in L2 motor neurons of <i>Smn</i><sup><i>2B/-</i></sup> SMA mice.

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    (A) ChAT and p53 immunostaining of the L2 spinal cord from control (Smn2B/+) and SMA (Smn2B/-) mice at P16. Scale bars: 100 μm. (B) ChAT and p53 immunostaining of L2 motor neurons from the same groups as in (A). Scale bars: 50 μm. (C) ChAT and phospho-p53S18 immunostaining of L2 motor neurons from the same groups as in (A). Scale bars: 50 μm. (D) Percentage of p53+ L2 motor neurons from the same groups as in (A). (E) Percentage of phospho-p53S18+ L2 motor neurons from the same groups as in (A). Data represents individual values, mean and SEM from 3 mice per group. Statistics were performed with two-tailed unpaired Student’s t-test. ** P (TIF)</p

    <i>Smn</i><sup><i>2B/-</i></sup> SMA mice display severe loss of proprioceptive synapses on motor neurons.

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    (A) Immunostaining of VGluT1+ synapses (grayscale) and ChAT+ motor neurons (blue) in the L2 spinal cord from control (Smn 2B/+) and SMA (Smn2B/-) mice at P16. Scale bars: 25μm. (B) Number of VGluT1+ synapses on the soma of L2 motor neurons from the same groups as in (A) at P16. The box-and-whiskers graph shows the individual values, median, interquartile range, minimum and maximum from 5 mice per group. Statistics were performed with two-tailed unpaired Student’s t-test. **** P < 0.0001.</p

    Smn deficiency induces p53 accumulation but not serine 18 phosphorylation in L1 motor neurons of <i>Smn</i><sup><i>2B/-</i></sup> SMA mice.

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    (A) ChAT and p53 immunostaining of the L1 spinal cord from control (Smn2B/+) and SMA (Smn2B/-) mice at P16. Scale bars: 100 μm. (B) ChAT and p53 immunostaining of L1 motor neurons from the same groups as in (A). Scale bars: 50 μm. (C) ChAT and phospho-p53S18 immunostaining of L1 motor neurons from the same groups as in (A). Scale bars: 50 μm. (D) Percentage of p53+ L1 motor neurons from the same groups as in (A). (E) Percentage of phospho-p53S18+ L1 motor neurons from the same groups as in (A). Data represents individual values, mean and SEM from 3 mice per group. Statistics were performed with two-tailed unpaired Student’s t-test. *** P < 0.001.</p

    Behavioral characterization of <i>Smn</i><sup><i>2B/-</i></sup> SMA mice.

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    (A) Body weight of control Smn2B/+ (n = 32) mice and Smn2B/- SMA mice (n = 31). Data represent mean and SEM. Statistics were performed with two-way ANOVA and Bonferroni’s multiple comparison test. ** P < 0.01; **** P < 0.0001. (B) Kaplan-Meier survival curves from the same experimental groups as in (A). Statistics were performed with Log-rank (Mantel-Cox) test. **** P < 0.0001. (C) Righting time from the same experimental groups shown in (A). Data represent mean and SEM. Statistics were performed with two-way ANOVA and Bonferroni’s multiple comparison test. Not Significant. (D) Time to fall in the hindlimb suspension test from the same experimental groups shown in (A). Data represent mean and SEM. Statistics were performed with two-way ANOVA and Bonferroni’s multiple comparison test. *** P < 0.001; **** P < 0.0001.</p
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