Skip to main content
. 2013 Oct 15;5(12):1871–1886. doi: 10.1002/emmm.201302979

Figure 6.

Mistargeting of lysosomal hydrolases leads to lysosomal dysfunction in MLII osteoblasts.

  1. Relative intracellular enzyme activity of lysosomal hydrolases β-hexosaminidase (β-hex) and β-galactosidase (β-gal) in primary cultured osteoblasts from MLII mice compared to WT. Data are shown as mean and SD (n = 3 primary cultures per group). *p (β-hex) = 0.0001, *p (β-gal) = 0.0002 for WT versus MLII by independent Student's t-test.

  2. Primary cultures of osteoblasts from MLII and WT mice were incubated with [35S]-methionine for 1 h and either harvested (0 h) or chased for 5 and 20 h, followed by immunoprecipitation of lysosomal proteases CtsZ and CtsD from cell extracts and media (p, precursor posttranslational modified by high-mannose type N-glycans; p′, precursor modified with N-glycans processed to the complex type form, m, mature form).

  3. Osteoblasts were incubated with [I125]-labelled arylsulphatase B (ASB) in the absence or presence of 10 mM mannose 6-phosphate (M6P). Cells were either harvested or chased for 3, 6 and 12 h. (p, precursor form; m, mature form).

Figure 6