Article
Pseudoachondroplasia is caused through both intra- and extracellular pathogenic pathways
2002-08-15
Abstract excerpt
Pseudoachondroplasia is a dominantly inherited chondrodysplasia associated with mutations in cartilage oligomeric matrix protein (COMP).Investigations into the pathogenesis of pseudoachondroplasia are hampered by its rarity.We developed a cell culture model by expressing mutant COMP in bovine primary chondrocytes using a gutless adenoviral vector.We show that mutant COMP exerts its deleterious effects through both...
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Identifiers and source
- Literature Corpus work
- 68e595d4-6c61-5eb8-b185-f4bda4123720
- DOI
- 10.1172/jci0214386
