Impaired osteoclastic bone resorption leads to osteopetrosis in cathepsin-K-deficient mice

DOI Web Site 被引用文献60件
  • Paul Saftig
    Zentrum Biochemie und Molekulare Zellbiologie, Abteilung Biochemie II, Universität Göttingen, Gosslerstrasse 12 d, 37073 Göttingen, Germany; Universität Bern, M.E. Müller-Institut für Biomechanik, Murtenstrasse 35, CH-3010 Bern, Switzerland; Department of Anatomy and Developmental Biology, University College London, Gower Street, London WC1E 6BT, United Kingdom; Institut für Biochemie, Universität Jena, Löbder Strasse 3, 07740 Jena, Germany; and Zentrum Radiologie, Universität Göttingen, Robert-Koch...
  • Ernst Hunziker
    Zentrum Biochemie und Molekulare Zellbiologie, Abteilung Biochemie II, Universität Göttingen, Gosslerstrasse 12 d, 37073 Göttingen, Germany; Universität Bern, M.E. Müller-Institut für Biomechanik, Murtenstrasse 35, CH-3010 Bern, Switzerland; Department of Anatomy and Developmental Biology, University College London, Gower Street, London WC1E 6BT, United Kingdom; Institut für Biochemie, Universität Jena, Löbder Strasse 3, 07740 Jena, Germany; and Zentrum Radiologie, Universität Göttingen, Robert-Koch...
  • Olaf Wehmeyer
    Zentrum Biochemie und Molekulare Zellbiologie, Abteilung Biochemie II, Universität Göttingen, Gosslerstrasse 12 d, 37073 Göttingen, Germany; Universität Bern, M.E. Müller-Institut für Biomechanik, Murtenstrasse 35, CH-3010 Bern, Switzerland; Department of Anatomy and Developmental Biology, University College London, Gower Street, London WC1E 6BT, United Kingdom; Institut für Biochemie, Universität Jena, Löbder Strasse 3, 07740 Jena, Germany; and Zentrum Radiologie, Universität Göttingen, Robert-Koch...
  • Sheila Jones
    Zentrum Biochemie und Molekulare Zellbiologie, Abteilung Biochemie II, Universität Göttingen, Gosslerstrasse 12 d, 37073 Göttingen, Germany; Universität Bern, M.E. Müller-Institut für Biomechanik, Murtenstrasse 35, CH-3010 Bern, Switzerland; Department of Anatomy and Developmental Biology, University College London, Gower Street, London WC1E 6BT, United Kingdom; Institut für Biochemie, Universität Jena, Löbder Strasse 3, 07740 Jena, Germany; and Zentrum Radiologie, Universität Göttingen, Robert-Koch...
  • Alan Boyde
    Zentrum Biochemie und Molekulare Zellbiologie, Abteilung Biochemie II, Universität Göttingen, Gosslerstrasse 12 d, 37073 Göttingen, Germany; Universität Bern, M.E. Müller-Institut für Biomechanik, Murtenstrasse 35, CH-3010 Bern, Switzerland; Department of Anatomy and Developmental Biology, University College London, Gower Street, London WC1E 6BT, United Kingdom; Institut für Biochemie, Universität Jena, Löbder Strasse 3, 07740 Jena, Germany; and Zentrum Radiologie, Universität Göttingen, Robert-Koch...
  • Winfried Rommerskirch
    Zentrum Biochemie und Molekulare Zellbiologie, Abteilung Biochemie II, Universität Göttingen, Gosslerstrasse 12 d, 37073 Göttingen, Germany; Universität Bern, M.E. Müller-Institut für Biomechanik, Murtenstrasse 35, CH-3010 Bern, Switzerland; Department of Anatomy and Developmental Biology, University College London, Gower Street, London WC1E 6BT, United Kingdom; Institut für Biochemie, Universität Jena, Löbder Strasse 3, 07740 Jena, Germany; and Zentrum Radiologie, Universität Göttingen, Robert-Koch...
  • Jörg Detlev Moritz
    Zentrum Biochemie und Molekulare Zellbiologie, Abteilung Biochemie II, Universität Göttingen, Gosslerstrasse 12 d, 37073 Göttingen, Germany; Universität Bern, M.E. Müller-Institut für Biomechanik, Murtenstrasse 35, CH-3010 Bern, Switzerland; Department of Anatomy and Developmental Biology, University College London, Gower Street, London WC1E 6BT, United Kingdom; Institut für Biochemie, Universität Jena, Löbder Strasse 3, 07740 Jena, Germany; and Zentrum Radiologie, Universität Göttingen, Robert-Koch...
  • Peter Schu
    Zentrum Biochemie und Molekulare Zellbiologie, Abteilung Biochemie II, Universität Göttingen, Gosslerstrasse 12 d, 37073 Göttingen, Germany; Universität Bern, M.E. Müller-Institut für Biomechanik, Murtenstrasse 35, CH-3010 Bern, Switzerland; Department of Anatomy and Developmental Biology, University College London, Gower Street, London WC1E 6BT, United Kingdom; Institut für Biochemie, Universität Jena, Löbder Strasse 3, 07740 Jena, Germany; and Zentrum Radiologie, Universität Göttingen, Robert-Koch...
  • Kurt von Figura
    Zentrum Biochemie und Molekulare Zellbiologie, Abteilung Biochemie II, Universität Göttingen, Gosslerstrasse 12 d, 37073 Göttingen, Germany; Universität Bern, M.E. Müller-Institut für Biomechanik, Murtenstrasse 35, CH-3010 Bern, Switzerland; Department of Anatomy and Developmental Biology, University College London, Gower Street, London WC1E 6BT, United Kingdom; Institut für Biochemie, Universität Jena, Löbder Strasse 3, 07740 Jena, Germany; and Zentrum Radiologie, Universität Göttingen, Robert-Koch...

書誌事項

公開日
1998-11-10
DOI
  • 10.1073/pnas.95.23.13453
公開者
Proceedings of the National Academy of Sciences

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説明

<jats:p> Cathepsin K is a recently identified lysosomal cysteine proteinase. It is abundant in osteoclasts, where it is believed to play a vital role in the resorption and remodeling of bone. Pycnodysostosis is a rare inherited osteochondrodysplasia that is caused by mutations of the cathepsin-K gene, characterized by osteosclerosis, short stature, and acroosteolysis of the distal phalanges. With a view to delineating the role of cathepsin K in bone resorption, we generated mice with a targeted disruption of this proteinase. Cathepsin-K-deficient mice survive and are fertile, but display an osteopetrotic phenotype with excessive trabeculation of the bone-marrow space. Cathepsin-K-deficient osteoclasts manifested a modified ultrastructural appearance: their resorptive surface was poorly defined with a broad demineralized matrix fringe containing undigested fine collagen fibrils; their ruffled borders lacked crystal-like inclusions, and they were devoid of collagen-fibril-containing cytoplasmic vacuoles. Assaying the resorptive activity of cathepsin-K-deficient osteoclasts <jats:italic>in vitro</jats:italic> revealed this function to be severely impaired, which supports the contention that cathepsin K is of major importance in bone remodeling. </jats:p>

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