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Updated: Jul 19, 2026
07:54
In vitro Synthesis of Native, Fibrous Long Spacing and Segmental Long Spacing Collagen
Published on: September 20, 2012
Collagen fibril arabesques in connective tissue disorders
Trinh Hermanns-Lê1, Gérald E Piérard
1Department of Dermatopathology, University Hospital of Liège, Liège, Belgium.
American Journal of Clinical Dermatology
|September 30, 2006
Summary
This study reveals novel collagen fibril shapes in Ehlers-Danlos syndrome (EDS) and related disorders, impacting tissue strength and cohesiveness.
Area of Science:
- Dermatology
- Biomedical Engineering
- Genetics
Background:
- Connective tissue disorders often present with ultrastructural abnormalities in collagen and elastic fibers.
- Ehlers-Danlos syndrome (EDS) is a group of inherited disorders affecting collagen, leading to hyperelasticity and tissue fragility.
Purpose of the Study:
- To investigate collagen fibril orientation and morphology in the dermis of patients with Ehlers-Danlos syndrome (EDS) and related conditions.
- To identify and document unusual collagen fibril shapes not previously described in the literature.
Main Methods:
- Utilized electron microscopy to examine dermal tissue samples.
- Focused analysis on collagen fibril orientation and the characterization of their shapes.
Main Results:
- Observed previously documented abnormal collagen fibril shapes characteristic of EDS and related syndromes.
- Identified and illustrated novel collagen fibril conformations, including hook-shaped, S-shaped, and ring-shaped structures.
Conclusions:
- The newly identified abnormal collagen fibril conformations in EDS and related disorders likely compromise tissue integrity.
- These ultrastructural changes may significantly influence the mechanical properties and cohesiveness of connective tissues, contributing to disease phenotypes.
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