Article
Characterization of the renal phenotype in a mouse model of Marfan syndrome.
Virchows Archiv : an international journal of pathology - 1 Oct 2004
Hartner Andrea, Eifert Timo, Haas Christian S, Tuysuz Cigdem, Hilgers Karl F, Reinhardt Dieter P, Amann Kerstin
Abstract excerpt
The microfibrillar protein fibrillin-1 is expressed abundantly in the vasculature and the glomerulus of the kidney. Mutations in the fibrillin-1 gene lead to Marfan syndrome. The most common complication of this disease is aortic dilatation due to elastic deficiencies of the vascular wall. Severa...
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