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. 2014 Feb 20;289(14):10211–10222. doi: 10.1074/jbc.M113.506246

FIGURE 4.

FIGURE 4.

HPβCD treatment results in reduced storage of ceroid lipopigment. A, confocal microscopy analysis of ceroid lipopigment (top, green) and TFEB (bottom, red) in LINCL patient-derived fibroblasts treated with 0.1, 1, and 10 mm HPβCD evaluated by detecting green autofluorescence and binding of an anti-TFEB antibody, respectively. The scale bar is 20 μm. UT, untreated. B, quantification of ceroid lipopigment autofluorescence in LINCL patient-derived fibroblasts treated as described in A. Representative fields containing ∼50 cells were analyzed. The scale bar is 20 μm. UT, untreated. Data are reported as the mean ± S.D. (p < 0.05; *, p < 0.01). C, confocal microscopy analysis of ceroid lipopigment (top, green) and TFEB (bottom, red) in LINCL fibroblasts treated with 1 mm HPβCD for 1, 3, and 7 days and evaluated as described in a. D and E, relative mRNA expression levels of representative genes of the lysosome-autophagy system in LINCL fibroblasts treated with 1 mm HPβCD for 3 days. GBA, HEXA, LAMP1, MAPLC3B, SQSTM1, and BECN1 mRNA expression levels were obtained as described in Fig. 1 (p < 0.01).