Ehlers-Danlos syndrome type IV (EDS IV) as model of a defective biopolymer composite material.
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.
Nemetschek
T
T
Department of Ultrastructure Research, University of Heidelberg, Germany.
Folkhard
W
W
Knörzer
E
E
Mosler
E
E
Nemetschek-Gansler
H
H
Koch
M H
MH
Meinel
A
A
Romen
W
W
D000328:Adult; D001011:Aorta / Q000032:analysis / Q000503:physiopathology; D001696:Biomechanical Phenomena; D003094:Collagen / Q000032:analysis / Q000378:metabolism*; D003238:Connective Tissue / Q000032:analysis / Q000503:physiopathology*; D003488:Cyanogen Bromide; D004535:Ehlers-Danlos Syndrome / Q000503:physiopathology*; D004549:Elastin / Q000378:metabolism*; D004591:Electrophoresis, Polyacrylamide Gel; D006801:Humans; D006909:Hydroxyproline / Q000032:analysis; D046911:Macromolecular Substances; D008297:Male; D008854:Microscopy, Electron; D010446:Peptide Fragments / Q000032:analysis; D013710:Tendons / Q000032:analysis / Q000503:physiopathology; D014961:X-Ray Diffraction
D046911:Macromolecular Substances; D010446:Peptide Fragments; D003094:Collagen; D004549:Elastin; D003488:Cyanogen Bromide; D006909:Hydroxyproline