Figure 3.
Acvr1G328V Causes Differentiation Arrest of Oligodendroglial Lineage Cells
(A) Heatmap depicting the relative expression of the top differentially expressed genes in the brainstem of postnatal day 7 (P7) pups.
(B) Expression of selected genes, measured by qPCR, in the brainstems from P7 pups.
(C) Immunofluorescence images showing CNP1 expression in the brainstem of mice at P21. Scale bars, 100 μm.
(D) Representative PDGFRA immunohistochemistry images in brainstem sections from mice at P14. Scale bars, 100 μm.
(E) Representative flow cytometry histograms, and quantification of the percentage of positive cells and mean fluorescence intensity, of PDGFRA antibody-stained brainstem cells from P7 pups. Each dot represents an individual animal; horizontal bars represent the mean.
(F) Representative immunohistochemistry images showing PDGFRA protein expression in four ACVR1 mutant DIPG samples. Scale bars, 50 μm.
(G) Expression of selected genes, measured by qPCR, in the brainstems of P7 pups.
(H) Expression of Pdgfra (left), and number of neurospheres generated, plotted as a function of the number of cells plated (right) in Acvr1+/+ neural stem cells transduced with lentiviruses encoding tdTomato, Ascl1, Sox11, or both Ascl1 and Sox11. n = 5 (left) and 3 (right) experiments.
In all panels, mean + SEM is shown. ∗p < 0.05, ∗∗p < 0.01, ∗∗∗p < 0.001; assessed by unpaired t test (B, E, and G), repeated-measures ANOVA with Tukey multiple comparisons tests (H). See also Figure S3 and Table S1.