Aberrant Phex function in osteoblasts and osteocytes alone underlies murine X-linked hypophosphatemia
J. Clin. Invest. Baozhi Yuan, et al. 118:722 doi:10.1172/JCI32702 [Go to this article.]

Figure 5
Bone histomorphology in normal, hyp-, Cre-Phex^Δflox/y, and OC-Cre-Phex^Δflox/y mice. (A) Goldner-stained sections of cortical bone reveal at low magnification an apparent increase in unmineralized osteoid (red-brown colored) in the hyp-, Cre-Phex^Δflox/y, and OC-Cre-Phex^Δflox/y mice, compared with that in normals. At higher magnification, the evident increased unmineralized osteoid in the cortical bone specimens from the hyp-, Cre-Phex^Δflox/y, and OC-Cre-Phex^Δflox/y mice appears comparable in magnitude. (B) The double-labeled bone specimens, viewed under fluorescent light, show normal mineralization in the normal mice, manifested by distinct dual labels deposited beneath narrow osteoid seams. In contrast, the bone sections from the hyp-, Cre-Phex^Δflox/y, and OC-Cre-Phex^Δflox/y mice have diffuse smudged fluorescent labels under widened osteoid seams, indicating a disorderly deposition of mineral characteristic of osteomalacia. The diffuse patchy double labels were too indistinct to permit quantitative assessment of the abnormal mineralization dynamics. (C) Quantitative histological exam of the Goldner-stained sections from a minimum of 6 animals in each group revealed significantly increased osteoid surface and osteoid volume in the hyp-, Cre-Phex^Δflox/y, and OC-Cre-PhexΔflox/y mice, as indicated by the asterisks denoting statistically significant values (***P < 0.001). In contrast, there was no significant difference in these values in the knockout models and the hyp-mice (denoted by the black columns), again providing evidence that the osteomalacia was of comparable magnitude in these animal models.