Summary
Study on connective tissue metabolism was conducted with a patient with Ehlers-Danlos syndrome (E-D) of Type J who visited our institute.
The conversion of procollagen into tropocollagen in the medium of cultured fibroblasts was assayed by the chase technique using3H-proline. The conversion was inhibited in the cultured fibroblasts of the patient.
The components of glycosaminoglycans in cultured medium and fibroblasts from E-D were within normal ranges, however, the ratio of glycoprotein to glycosaminoglycans of intra-and extra-cellular fractions of E-D fibroblasts was higher than the normal one.
These findings suggest that the insufficient maturation of collagen fiber may be considered fundamental disorders of E-D.
Zusammenfassung
Der Bindegewebsstoffwechsel wird bei einem Patienten mit einem Ehlers-Danlos-Syndrom des Typs I untersucht.
Die Umwandlung von Prokollagen in Tropokollagen in die Fibroblastenkultur wurde mittels3H-Prolin durchgeführt. Der Prozeß der Umwandlung wird in der Fibroblastenkultur des Patienten gehemmt.
Die Glykosaminoglykane im Kulturmedium und die Fibroblasten des Patienten zeigten keine Abweichung von der Norm, jedoch war das Verhältnis von Glykoproteinen zu Glykosaminoglykanen der intra- und extracellulären Fraktion der Fibroblasten des Patienten höher als bei normalen Patienten.
Diese Ergebnisse lassen den Hinweis zu, daß eine ungenügende Reife der Kollagenfaser eine der wesentlichsten Ursachen des Ehlers-Danlos-Syndroms ist.
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Shinkai, H., Hirabayashi, O., Tamaki, A. et al. Connective tissue metabolism in culture fibroblasts of a patient with Ehlers-Danlos syndrome type I. Arch. Derm. Res. 257, 113–123 (1976). https://doi.org/10.1007/BF00558084
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DOI: https://doi.org/10.1007/BF00558084