The Ehlers-Danlos syndrome: on beyond collagens
J. Clin. Invest. Jau-Ren Mao, et al. 107:1063 doi:10.1172/JCI12881 [
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Figure 2The collagen fibril and EDS. (
a) Normal collagen fibrils are of uniform size and spacing. Fibrils from a patient with dermatosparaxis (
b) show dramatic alterations in fibril morphology with severe effects on tensile strength of connective tissues. Most fibrils from a patient with classical EDS (
c) are normal in appearance. Composite fibrils (arrows) are typically rare. Fibrils from a TNX-deficient patient (
d) are uniform in size and no composite fibrils are seen. When compared with normal skin (
e), TNX-null fibrils are less densely packed and not as well aligned to neighboring fibrils. In normal skin (
e) and cornea (
f), fibrils are deposited in tissue-specific patterns. In skin, bundles of fibrils are oriented in different directions to resist forces in multiple axes. In cornea, orthogonal arrays allow maximal transparency.
