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. 2019 Sep 10;86(5):780–792. doi: 10.1002/ana.25585

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© 2019 The Authors. Annals of Neurology published by Wiley Periodicals, Inc. on behalf of American Neurological Association.

This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.

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Mouse white matter (WM) and gray matter (GM) astrocytes are differentially affected by vanishing white matter (VWM) mutation. (A, B) Immunostaining for cytoplasmic cell surface marker ezrin of mouse induced pluripotent stem cell (miPSC)‐derived WM (A) and GM astrocytes (B) showed morphological differences between the cells. (C–H) To assess functional defects of VWM mouse WM and GM astrocytes, wild‐type (wt) mouse primary oligodendrocyte precursor cells were cultured in conditioned medium collected from wt (n = 3), 2b5 ^ho (n = 4), and 2b4 ^ho 2b5 ^ho (n = 4) iPSC‐derived GM or WM astrocytes (respectively GM and WM in I–K). Oligodendrocyte maturation was quantified as the percentage of myelin basic protein (MBP)‐positive oligodendrocytes of the number of Olig2‐positive cells. (I, J) This percentage was significantly decreased in 2b4 ^ho 2b5 ^ho GM medium compared to wt WM medium and 2b4 ^ho 2b5 ^ho GM medium (I), whereas the percentage of Olig2‐positive cells was unchanged between the conditions (J). To assess apoptosis in the oligodendrocyte cultures, a cleaved caspase 3 (CC3) assay was conducted in oligodendrocyte cultures with conditioned medium of GM and WM astrocytes of wt (n = 4), 2b5 ^ho (n = 4), and 2b4 ^ho 2b5 ^ho (n = 4). (K) The percentage of CC3‐positive cells of 4,6‐diamidino‐2‐phenylindole (DAPI)‐positive cells was significantly decreased in VWM compared to control WM cultures. *p < 0.05, **p < 0.01. Scale bars = 50μm. Bars in I–K represent mean ± standard error of the mean. (L) Volcano plot of differential expression analysis between wt (n = 4) and 2b4 ^ho 2b5 ^ho (n = 4) mouse WM astrocytes with significant differentially expressed genes (DEGs). Significant DEGs are colored in red and are labeled. (M) Significantly enriched Gene Ontology terms for DEGs in different categories.