Spondyloepiphyseal dysplasia congenita: morphological and biochemical studies in cultured chondrocytes

ABSTRACT Human chondrocytes dedifferentiate when they grow in monolayer cultures and produce type I collagen. Re-differentiation is possible by culturing them in an agarose-gel. To

characterize the modulated cells in more detail, ultrastructural and biochemical studies were performed in chondrocytes suspended in agarose for 2 weeks. It was shown that their

ultrastructural characteristics were indistinguishable from those in tissue cells. Glycosaminoglcan (GAG) composition, electrophoretic mobility and hydrodynamic size of proteoglycans were

identical in tissue and in cultured cells. Chondrocytes from a patient with spondyloepiphyseal dysplasia congenita showed characteristic ultrastructural abnormalities both in biopsied

cartilage and in cultured cells, most notably a dilated rough endoplasmic reticulum containing fine granular material. Proteoglycans produced by cultured cells contained abnormally high

amounts of dermatan sulfate. The GAG chains were shorter than tose produced by normal cells. It remains to be established how these abnormalities are related to defects in collagen type II

production that have been shown to exist in spondyloepiphyseal dysplasia congenita. ARTICLE PDF AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Univ.-Kinderklinik, Langenbeckstrabe 1, Mainz,

6500, FRG M Beck, K Becker & J Spranger Authors * M Beck View author publications You can also search for this author inPubMed Google Scholar * K Becker View author publications You can

also search for this author inPubMed Google Scholar * J Spranger View author publications You can also search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints and

permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Beck, M., Becker, K. & Spranger, J. SPONDYLOEPIPHYSEAL DYSPLASIA CONGENITA: MORPHOLOGICAL AND BIOCHEMICAL STUDIES IN CULTURED

CHONDROCYTES. _Pediatr Res_ 26, 515 (1989). https://doi.org/10.1203/00006450-198911000-00093 Download citation * Issue Date: 01 November 1989 * DOI:

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