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© 1995 British Society for Rheumatology

research-article

ABNORMAL COLLAGEN CROSS-LINKING IN THE CARTILAGE OF A DIASTROPHIC DYSPLASIA PATIENT

A. J. BAILEY*,, T. J. SIMS*, V. STANESCU{dagger}, P. MAROTEAUX{dagger} and R. STANESCU{dagger}

*Muscle and Collagen Research Group, University of Bristol Longford, Bristol
{dagger}Clinique Maurice Lamy, Hôpital des Enfants Malades Paris Cédex 15, France

Correspondence to: Correspondence to: A. J. Bailey, Muscle and Collagen Research Group, Division of Molecular and Cellular Biology, University of Bristol, Langford, Bristol BS18 7DY.

The abnormal organization of the cartilage collagen in disatrophic dysplasia is not generally reflected in the levels of the major stabilizing cross-link, hudroxylysyl–pyridinoline. However, is one case there was a marked decrease in the pyridinoline concomitant with the appearance of two unknown components in the cross-link region of the chromatogra. A decrease in cross-link of this magnitude could lead to mechanically weakened cartilage. Insufficient material was available to characterise these unknown components. The disorganization of cartilage in some cases of disatrophic dysplasia could therefore de due to post-translational modification, including defective cross-linking

KEY WORDS: Collagen, Cross-linking, Cartilage, Dysplasia


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