Figure 7.

Bone mineralization and body composition of the KO mice at 5 mo of age. Because the KO mouse phenotype is neonatally lethal, all mice were treated with 3 weekly injections of 20NHS PEG-CBS (SC, 7.5 mg/kg) from d 2 of age to allow their survival into adulthood. At 35 d of age, the mice were randomized into 3 groups: healthy control mice consisting of heterozygous KO mice (n = 4 M + 6 F; black bars); affected KO mice (n = 5 M + 13 F; white bars), which were left untreated from d 35 on; and treated KO mice (n = 5 M + 7 F; hatched bars) continued receiving treatment as during the initial 5 wk. The DXA scans were performed when the mice reached ∼5 mo of age. Individual panels show bone mineral density (A), bone mineral content (B), total mass (C), lean mass (D), and fat content (E). Bars represent an average value from individual mice. Error bars indicate sem. *P < 0.05, **P < 0.01, ***P < 0.001.