Cartilage oligomeric matrix protein-deficient mice have normal skeletal development.

Liz Svensson, Attila Aszodi, Dick Heinegård, Ernst B Hunziker, Finn P Reinholt, Reinhard Fässler, Åke Oldberg

Forskningsoutput: TidskriftsbidragArtikel i vetenskaplig tidskriftPeer review

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Sammanfattning

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.
Originalspråkengelska
Sidor (från-till)4366-4371
TidskriftMolecular and Cellular Biology
Volym22
Nummer12
DOI
StatusPublished - 2002

Bibliografisk information

The information about affiliations in this record was updated in December 2015.
The record was previously connected to the following departments: Experimental Pathology (013031100), Cell and Matrix Biology (LUR000002), Åke Oldberg´s group (013212049), Pathology, (Lund) (013030000), Connective Tissue Biology (013230151)

Ämnesklassifikation (UKÄ)

  • Cell- och molekylärbiologi

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