Fibrillin-containing microfibrils: structure and function in health and disease.
ABSTRACT Fibrillin-containing microfibrils are a unique class of connective tissue macromolecules whose critical contribution to the establishment and maintenance of diverse extracellular matrices was underlined by the recent linkage of their principal structural component fibrillin to Marfan syndrome, a heritable disorder with pleiotrophic connective tissue manifestations. The complexity of the structure: function relationships of these macromolecules was highlighted by the recent elucidation of the primary structure of fibrillin and characterisation of fibrillin mutations in Marfan patients. This review examines current understanding of the expression and assembly of fibrillin and describes new approaches which are now being applied to elucidate the many outstanding structural, organisational and functional aspects of the fibrillin-containing microfibrils.
- American Journal of Pathology - AMER J PATHOL. 01/2000; 156(3):925-938.
Article: Marfan syndrome.New England Journal of Medicine 10/1990; 323(14):987-9. · 54.42 Impact Factor