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. 2014 Jun 4;137(7):1894–1906. doi: 10.1093/brain/awu114

Figure 4.

Figure 4

Markers of endoplasmic reticulum stress are increased in cultured motor neurons from SBMA mice. (A) The PERK-activated pathway of the unfolded protein response was examined to establish the role of endoplasmic reticulum stress in motor neurons. Cultures were immunostained for a neuronal marker (β-III tubulin, green), a nuclear marker (DAPI, blue) and endoplasmic reticulum stress markers BiP, ATF4 or CHOP (all red). In all cases immunoreactivity for the endoplasmic reticulum stress markers appeared to be elevated in AR100 motor neurons compared to wild-type (WT) controls. Scale bars in images are 30 µm. (B) Purified primary motor neuron cultures from at least three different wild-type and AR100 embryos were analysed by western blot to quantify the levels of BiP, ATF4 and CHOP proteins. (C) Densitometry showed that the expression levels of BiP, ATF4 and CHOP were significantly higher in purified AR100 motor neurons than in wild-type controls (n = 3, P = 0.05, 0.047 and 0.008 respectively, t-test, n = number of mice). Error bars show the SEM. *P < 0.05. AU = arbitrary units.