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Molecular and Cellular Biology, February 2004, p. 1691-1699, Vol. 24, No. 4
0270-7306/04/$08.00+0     DOI: 10.1128/MCB.24.4.1691-1699.2004
Copyright © 2004, American Society for Microbiology. All Rights Reserved.

Matrilin-3 Is Dispensable for Mouse Skeletal Growth and Development

Yaping Ko,¹ Birgit Kobbe,^1, Claudia Nicolae,^2, Nicolai Miosge,³ Mats Paulsson,¹ Raimund Wagener,¹ and Attila Aszódi^2*

Center for Biochemistry, Medical Faculty, University of Cologne, D-50931 Cologne,¹ Center of Anatomy, Department of Histology, University of Göttingen, D-37075 Göttingen,³ Department for Molecular Medicine, Max Planck Institute for Biochemistry, D-82152 Martinsried, Germany²

Received 25 August 2003/ Accepted 14 November 2003

Matrilin-3 belongs to the matrilin family of extracellular matrix (ECM) proteins and is primarily expressed in cartilage. Mutations in the gene encoding human matrilin-3 (MATN-3) lead to autosomal dominant skeletal disorders, such as multiple epiphyseal dysplasia (MED), which is characterized by short stature and early-onset osteoarthritis, and bilateral hereditary microepiphyseal dysplasia, a variant form of MED characterized by pain in the hip and knee joints. To assess the function of matrilin-3 during skeletal development, we have generated Matn-3 null mice. Homozygous mutant mice appear normal, are fertile, and show no obvious skeletal malformations. Histological and ultrastructural analyses reveal endochondral bone formation indistinguishable from that of wild-type animals. Northern blot, immunohistochemical, and biochemical analyses indicated no compensatory upregulation of any other member of the matrilin family. Altogether, our findings suggest functional redundancy among matrilins and demonstrate that the phenotypes of MED disorders are not caused by the absence of matrilin-3 in cartilage ECM.

B.K. and C.N. contributed equally to this work.

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