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. 2018 Apr 29;19(5):1326. doi: 10.3390/ijms19051326

Figure 2.

Figure 2

Diagrammatic representation of the developmental changes in AChR composition in humans and mice. Differences between human and mouse expression of ε- and γ-AChR are highlighted and consequences for AChR-deficiency CMS patients and mouse models of AChR-deficiency are compared. Mouse model expressing human γ-AChR in ε-AChR knockout background provides robust model of human CMS phenotype.