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. Author manuscript; available in PMC: 2015 Aug 14.
Published in final edited form as: Chem Biol. 2014 Aug 14;21(8):967–976. doi: 10.1016/j.chembiol.2014.06.008

Figure 2.

Figure 2

ERdj3 is a proteostasis network component of mutant GCase. (A) Silencing ERdj3 increased the endo H-resistant glycoform of L444P GCase in fibroblasts. Quantification of endo H-resistant L444P GCase bands is shown below. (B) Silencing ERdj3 significantly increased L444P GCase activity in fibroblasts. (C) Silencing ERdj3 enhanced the lysosomal trafficking of L444P GCase, as assessed by indirect immunofluorescence microscopy. (D) Silencing ERdj3 increased the endo H-resistant glycoform of N370S GCase in fibroblasts. Quantification of endo H-resistant N370S GCase bands is shown below. (E) Silencing ERdj3 significantly increased N370S GCase activity in fibroblasts. The data in (A), (B), (D) and (E) are reported as mean ± SD (n = 3 for A and D, n = 8 for B and E). Statistical significance was calculated using a two-tailed Student’s t-test, * p < 0.01. See also Figures S2 and S3.