Ehlers-Danlos Syndrome Type-Iv (Eds-Iv) As Model Of A Defective Bio-Polymer Composite-Material
CONNECTIVE TISSUE RESEARCH(1989)
摘要
The connective tissue of a lethal EDS IV case was investigated for the reasons of the manifested disturbances of the arterial wall. This functional disorder was attributed to the mechanical decoupling of elastin and collagen, with the premise of a composite material consisting of cellular, fibrillar, lamellar and other matrix components. A conceivable relation between the manifested deficiency of type III collagen and a disturbed anchoring of elastin is shown. These findings are supported by biochemical, morphological, x-ray and mechanical data.
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关键词
composite material,synchrotron radiation,structural dynamics,x ray diffraction
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