Figure 5.
Three-dimensional micro-CT reconstructions of knee joints of wild-type, untreated MPS VII, and PerT-GUS treated MPS VII mice intraperitoneally (IP) (IP 2 mg/kg ERT started at day 2-3 and continued weekly until the autopsy). Each picture shows unsectioned bone (left side) or sagittal-sectioned bone (right side). Cross sections are sagittal through the midlines. The long arrows identify areas of thickened cortical bone. The short arrows identify abnormal exophytic bone formations on articular surfaces. Ages of wild-type and untreated MPS VII mice are 5, 23, and 36 weeks old. Ages of 2 mg/kg PerT-GUS treated mice are 27, 41, and 57 weeks-old.
Adapted from Rowan DJ, Tomatsu S, Grubb JH, et al. Long circulating enzyme replacement therapy rescues bone pathology in mucopolysaccharidosis VII murine model. Mol Genet Metab 2012 107(1-2):161-72.