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. 2009 Jul 9;28(15):2244–2258. doi: 10.1038/emboj.2009.159

Figure 7.

Figure 7

Jumpy R336Q mutant associated with centronuclear myopathy is defective in inhibiting autophagy. (A–K) C2C12 cells were transfected for 48 h with YFP, YFP-Jumpy WT (Jumpy WT), YFP-Jumpy C330S (Jumpy CS), YFP-Jumpy Y462C (Jumpy YC) or YFP-Jumpy R336Q (Jumpy RQ), fixed and immunostained with anti-p62 antibody (red). p62 puncta were quantitated by confocal fluorescence microscopy. Representative confocal images of YFP (A), YFP-Jumpy WT (B), YFP-Jumpy C330S (C), YFP-Jumpy Y462C (D) and YFP-Jumpy R336Q (E) transfected cells, immunostained for p62 (F), (G), (H), (I) and (J), respectively. Scale bars, 5 μm. Quantitation of p62 puncta per cell (K). Bars, s.e.m. (n=3 independent experiments, 30 cells per experiments). *P<0.05 (t-test), ns: nonsignificant. (L) HeLa cells were transfected for 24 h with YFP, YFP-Jumpy WT (Jumpy WT) or YFP-Jumpy R336Q (Jumpy RQ), incubated with or without 50 ng/μl rapamycin (BafA1 (100 nM) was present in both control and rapamycin treated cells) for 2 h, lysed and analysed for LC3, YFP and actin by immunoblotting. Densitometric LC3-II/actin ratios are shown underneath the blot.