<p>Each panel presents GC species levels in mice having the indicated <i>Gba1</i> mutation: D409H (9H/9H and 9H;C*), V394L (4L/4L and 4L;C*), D409V (9V/9V, 9V/null, and 9V;C*) and N370S (0S/0S and 0S;C*). The panels are grouped to reflect the various mutations to facilitate comparison of the GC levels. The ordinates differ to facilitate visualizing the differing levels of GC. Age-matched WT tissues were controls for each analysis. 4L;C* lung at 6 wks was compared to WT at 9 wks. Each GC species level in the mutant was analyzed relative to age-matched WT using Student’s t-test. *, p<0.05, **, p<0.01, ***, p<0.0001, ****, p<0.00001. The results and error bars are the mean+S.E. (n = 3–4 mice).</p
<p>(A) Representative images of BrdU (green) and NeuN (red) staining in the GCL of AC3<sup>+/+</sup>...
<p>(A) Mice (n = 12/group) were infected i.n. with 10<sup>4</sup> PFU of V<sub>K627</sub> (▪), rV<su...
<p>A. Bright field image of the inflated lung from <i>Gpr116</i> WT, heterozygous and knockout litte...
<p>The fold changes of GC species and glucosylsphingosine levels in individual mutant mice were age-...
<p>(A) The mean proportion of each GC species relative to total GC (6 GC species) in age-matched <i>...
a<p># indicates significantly changed GC species level relative to WT. Data present mean of 3–4 mice...
<p>Each graph depicts glucosylsphingosine levels in four tissues from mice having the same <i>Gba 1<...
<p>(A) Spleen GC level in 4L;C* mice was not different from that in the WT. GS level was accumulated...
<p>All GC species in WT mice showed age-dependent decrease with age (from <1 d, 6–9 wks to 52 wks) i...
<p>(A) Tissue GlcCer species in the lungs, livers, spleens and brains from 9-wk of WT, WT+GCStg, 9V/...
<p>Total GC levels in liver, lung, and spleen from 9V/null (red), 9H/9H (violet), 9V/9V (green), and...
<p>(A) GlcCer concentration in visceral tissues. The lungs, livers and spleens from 9-, 18-, 28- and...
Gaucher disease results from GBA1 mutations that lead to defective acid β-glucosidase (GCase) mediat...
Gaucher disease results from GBA1 mutations that lead to defective acid b-glucosidase (GCase) mediat...
<p>Panel A. A box-and-whisker plot of disease activity index of DKO mice grouped by strain backgroun...
<p>(A) Representative images of BrdU (green) and NeuN (red) staining in the GCL of AC3<sup>+/+</sup>...
<p>(A) Mice (n = 12/group) were infected i.n. with 10<sup>4</sup> PFU of V<sub>K627</sub> (▪), rV<su...
<p>A. Bright field image of the inflated lung from <i>Gpr116</i> WT, heterozygous and knockout litte...
<p>The fold changes of GC species and glucosylsphingosine levels in individual mutant mice were age-...
<p>(A) The mean proportion of each GC species relative to total GC (6 GC species) in age-matched <i>...
a<p># indicates significantly changed GC species level relative to WT. Data present mean of 3–4 mice...
<p>Each graph depicts glucosylsphingosine levels in four tissues from mice having the same <i>Gba 1<...
<p>(A) Spleen GC level in 4L;C* mice was not different from that in the WT. GS level was accumulated...
<p>All GC species in WT mice showed age-dependent decrease with age (from <1 d, 6–9 wks to 52 wks) i...
<p>(A) Tissue GlcCer species in the lungs, livers, spleens and brains from 9-wk of WT, WT+GCStg, 9V/...
<p>Total GC levels in liver, lung, and spleen from 9V/null (red), 9H/9H (violet), 9V/9V (green), and...
<p>(A) GlcCer concentration in visceral tissues. The lungs, livers and spleens from 9-, 18-, 28- and...
Gaucher disease results from GBA1 mutations that lead to defective acid β-glucosidase (GCase) mediat...
Gaucher disease results from GBA1 mutations that lead to defective acid b-glucosidase (GCase) mediat...
<p>Panel A. A box-and-whisker plot of disease activity index of DKO mice grouped by strain backgroun...
<p>(A) Representative images of BrdU (green) and NeuN (red) staining in the GCL of AC3<sup>+/+</sup>...
<p>(A) Mice (n = 12/group) were infected i.n. with 10<sup>4</sup> PFU of V<sub>K627</sub> (▪), rV<su...
<p>A. Bright field image of the inflated lung from <i>Gpr116</i> WT, heterozygous and knockout litte...