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Molecular and Cellular Biology, June 2002, p. 4366-4371, Vol. 22, No. 12
0270-7306/02/$04.00+0     DOI: 10.1128/MCB.22.12.4366-4371.2002
Copyright © 2002, American Society for Microbiology. All Rights Reserved.

Cartilage Oligomeric Matrix Protein-Deficient Mice Have Normal Skeletal Development

Liz Svensson,1,{dagger} Attila Aszódi,2,{ddagger} Dick Heinegård,1 Ernst B. Hunziker,3 Finn P. Reinholt,4 Reinhard Fässler,2,{ddagger} and Åke Oldberg1*

Department of Cell and Molecular Biology, BMC, University of Lund, S-221 84 Lund,1 Department of Experimental Pathology, University Hospital, S-221 85 Lund, Sweden,2 M. E. Muller-Institute for Biomechanics, University of Bern, CH-3010 Bern, Switzerland,3 Laboratory for Electron Microscopy, Institute/Department of Pathology, Rikshospitalet, University Hospital, NO-0027 Oslo, Norway4

Received 12 November 2001/ Returned for modification 14 January 2002/ Accepted 5 March 2002

Cartilage oligomeric matrix protein (COMP) belongs to the thrombospondin family and is a homopentamer primarily expressed in cartilage. Mutations in the COMP gene result in the autosomal dominant chondrodysplasias pseudoachondroplasia (PSACH) and some types of multiple epiphyseal dysplasia (MED), which are characterized by mild to severe short-limb dwarfism and early-onset osteoarthritis. We have generated COMP-null mice to study the role of COMP in vivo. These mice show no anatomical, histological, or ultrastructural abnormalities and show none of the clinical signs of PSACH or MED. Northern blot analysis and immunohistochemical analysis of cartilage indicate that the lack of COMP is not compensated for by any other member of the thrombospondin family. The results also show that the phenotype in PSACH/MED cartilage disorders is not caused by the reduced amount of COMP.

* Corresponding author. Mailing address: Department of Cell and Molecular Biology, University of Lund, BMC, C 13, S-221 84 Lund, Sweden. Phone: 46 46 2228577. Fax: 46 46 2223128. E-mail: ake.oldberg{at}medkem.lu.se.

{dagger} Present address: Novo Nordisk Scandinavia AB, 202 15 Malmö, Sweden.

{ddagger} Present address: Department of Molecular Medicine, Max Planck Institute for Biochemistry, 82152 Martinsried, Germany.

Molecular and Cellular Biology, June 2002, p. 4366-4371, Vol. 22, No. 12
0022-538X/02/$04.00+0     DOI: 10.1128/MCB.22.12.4366-4371.2002
Copyright © 2002, American Society for Microbiology. All Rights Reserved.



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