FIGURE

Figure 5

ID

ZDB-FIG-210512-55

Publication

Sofou et al., 2021 - Bi-allelic VPS16 variants limit HOPS/CORVET levels and cause a mucopolysaccharidosis-like disease

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Figure 5

Analysis of lysosomal distribution and function in fibroblasts

Confocal micrographs of fibroblasts transduced with control or VPS16‐expressing lentivirus and labeled with LysoTracker. Scale bar, 20 μm.

Quantification of LysoTracker‐stained puncta number and intensities (ca 80 cells analyzed for each of n = 3 biological replicates).

C, D

Representative immunoblots (bottom) and summary quantifications (top) of the levels of LAMP1 (C) and LAMP2 (D) in fibroblasts, normalized to levels of actin (ACTB) and expressed as % of controls (n = 3 biological replicates).

Analysis of lysosomal Cathepsin D (CtsD) processing by immunoblotting. Immunoblot to detect CtsD isoforms (left) and quantification (right) of processing, calculated as the percentage of CtsD divided by the sum of all isoforms (n = 3 biological replicates).

Data information: Bar graphs represent data as mean ±SEM. Statistical comparisons between the indicated groups by two‐tailed unpaired Student’s t‐tests (panel E) or one‐way ANOVA with Holm–Sidak’s multiple comparisons tests (panel B). *P < 0.05; **P < 0.01.

Expression Data

Expression Detail

Antibody Labeling

Phenotype Data

Phenotype Detail

Acknowledgments

This image is the copyrighted work of the attributed author or publisher, and ZFIN has permission only to display this image to its users. Additional permissions should be obtained from the applicable author or publisher of the image. Full text @ EMBO Mol. Med.