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Human Molecular Genetics, 1999, Vol. 8, No. 10 1839-1846
© 1999 Oxford University Press

Review

Osteopetrosis and osteoporosis:two sides of the same coin

Francesca Lazner, Maxine Gowen1, Durda Pavasovic and Ismail Kola+

Centre for Functional Genomics and Human Disease, Instituteof Reproduction and Development, Monash University, Monash MedicalCentre, 246 Clayton Road, Clayton, Victoria 3168, Australia and 1Department of Bone and CartilageBiology, SmithKline Beecham Pharmaceuticals, King of Prussia, PA19406, USA

ABSTRACT

Together, osteoporosis and osteopetrosis comprise asubstantial proportion of the bone diseases that severely affecthumans. In order to understand and effectively treat these disorders,an understanding of the mechanisms controlling bone remodellingis essential. While numerous animal models of bone disease have beengenerated, the lack of correlation between these animal models andhuman disease has limited their utility in terms of defining therapeuticstrategies. The generation and analysis of cathepsin K knockoutmice has resulted in a model for pycnodysostosis, a rare human osteopetroticdisease, and is now providing considerable insights into both osteoclastfunction and potential therapeutic strategies for the treatmentof bone disease. This review highlights the importance of genessuch as cathepsin K in understanding bone remodelling and illustratesa new trend towards understanding bone disease as a complete entityrather than as a series of unrelated disorders.

FOOTNOTES

+ Towhom correspondence should be addressed. Tel: +61 3 95947202; Fax: +61 3 9594 7212; Email: ismailko@silas.cc.monash.edu.au


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