Determination of bone markers in pycnodysostosis: effects of cathepsin K deficiency on bone matrix degradation.

@article{Nishi1999DeterminationOB,
  title={Determination of bone markers in pycnodysostosis: effects of cathepsin K deficiency on bone matrix degradation.},
  author={Yoshikazu Nishi and Lynne M Atley and D E Eyre and Jacob G. Edelson and Andrea Superti-Furga and Tomoh Yasuda and Robert J Desnick and Bruce D Gelb},
  journal={Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research},
  year={1999},
  volume={14 11},
  pages={1902-8}
}
Pycnodysostosis (Pycno) is an autosomal recessive osteosclerotic skeletal dysplasia that is caused by the markedly deficient activity of cathepsin K. This lysosomal cysteine protease has substantial collagenase activity, is present at high levels in osteoclasts, and is secreted into the subosteoclastic space where bone matrix is degraded. In vitro studies… CONTINUE READING