The high content of collagen V in the cornea accounts for the unique small diameter of corneal fibrils 6, 7. Collagen V is responsible for the formation of heterotypic fibrils together with collagen I 5, 6. On a molecular level, classic type EDS results from a mutation in COL5A1 and COL5A2.Ĭollagen V is present in the corneal stroma and makes approximately 10 to 20% of its collagen content, see review 4. Corneal thinning is a common finding in classic type EDS 1, 2, along with a steep cornea, but not a higher incidence of keratoconus 3. Results presented here support clinical findings, in which thin corneas with global ultrastructural alterations maintain a normal corneal shape.Įhlers–Danlos syndrome (EDS) is an autosomal dominant connective tissue disease primarily affecting skin and joints, and generally characterized by joint hypermobility, skin hyperextensibility and tissue fragility. We propose that disturbed collagen fibril structure in Col5a1 +/− corneas affects the viscoelastic properties. SHG microscopy showed differences in forward and backward scattered signal indicating abnormal collagen fibrils in Col5a1 +/− corneas. In contrast, in stress relaxation tests, Col5a1 +/− corneas experienced a stronger relaxation (55% vs 50%, p = 0.01). In a total of 28 eyes from a Col5a1 + /− mouse model and wild-type C57BL/6 littermates (wt), Col5a1 +/− corneas were thinner when compared to wt, (125 ± 11 vs 148 ± 10 μm, respectively, p < 0.001). 2D-extensometry measurements was conducted invasively consisting of a pre-conditioning cycle, a stress-relaxation test and a rupture test. Quasi-static Optical Coherence Elastography (OCE) was conducted non-invasively during ambient pressure modulation by − 3 mmHg. Here we quantify corneal biomechanical properties in an adult classic EDS mouse model using two different measurement approaches suited for murine corneal mechanical characterization and relate differences to stromal structure using Second Harmonic Generation (SHG) microscopy. Ehlers–Danlos syndrome (EDS) is a genetic disease leading to abnormalities in mechanical properties of different tissues.
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