Figure EV2. Compromised clearance of damaged lysosomes in p97 mutant‐expressing cells, and vacuolated muscle fibers from a p97 patient are positive for Gal3 and LAMP2 (related to Fig 2).
- Induction and expression levels of myc‐tagged p97 and variants in cells used in Fig 2B were determined via immunoblotting.
- Quantification of LC3‐II level normalized to GAPDH from three independent experiments (mean ± SD, Student's t‐test). LC3‐II level in U2OS cells treated with doxycycline (U2OS‐) were set to fold change = 1. *P < 0.05; **P < 0.01; ***P < 0.001.
- Skeletal muscle biopsy from a p97‐R155H patient. Serial sections were immunostained for Gal3 (red) and LAMP2 (green) and demonstrate colocalization in areas of lysosomal membrane accumulation within vacuolated fibers. Scale bar, 25 μm.
Source data are available online for this figure.