Ehlers-Danlos Syndrome Type IV (EDS IV) as Model of a Defective Biopolymer Composite Material

Th. Nemetschek Institute of Pathology, Department of Ultrastructure Research, University of Heidelberg, D-6900, Heidelberg, Germany

W. Folkhard Institute of Pathology, Department of Ultrastructure Research, University of Heidelberg, D-6900, Heidelberg, Germany

E. Knorzer Institute of Pathology, Department of Ultrastructure Research, University of Heidelberg, D-6900, Heidelberg, Germany

E. Mosler Institute of Pathology, Department of Ultrastructure Research, University of Heidelberg, D-6900, Heidelberg, Germany

H. Nemetschek-Gansler Institute of Pathology, Department of Ultrastructure Research, University of Heidelberg, D-6900, Heidelberg, Germany

M.H. J. Koch European Molecular Biology Laboratory c/o DESY, D-2000, Hamburg, 52, Germany

A. Meinel Central Hospital, Department of Surgery, D-6972, Tauberbischofsheim, Germany

W. Romen Institute of Pathology, Caritas Hospital, D-6990, Bad Mergentheim, Germany

show all

Abstract

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.

Key Words:

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Order Reprints Request Corporate Permissions

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

Request Academic Permissions

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.

Ehlers-Danlos Syndrome Type IV (EDS IV) as Model of a Defective Biopolymer Composite Material

Information for

Open access

Opportunities

Help and information

Ehlers-Danlos Syndrome Type IV (EDS IV) as Model of a Defective Biopolymer Composite Material

Abstract

Reprints and Corporate Permissions

Academic Permissions

Related research

To cite this article:

Download citation

Information for

Open access

Opportunities

Help and information

Keep up to date

Your download is now in progress and you may close this window

Login or register to access this feature