FIBRILLIN-CONTAINING MICROFIBRILS - STRUCTURE AND FUNCTION IN HEALTH AND DISEASE

Fibrillin-containing microfibrils are a unique class of connective tis sue macromolecules whose critical contribution to the establishment an d maintenance of diverse extracellular matrices was underlined by the recent linkage of their principal structural component fibrillin to Ma rfan syndrome, a heritable disorder with pleiotrophic connective tissu e manifestations. The complexity of the structure: function relationsh ips of these macromolecules was highlighted by the recent elucidation of the primary structure of fibrillin and characterisation of fibrilli n mutations in Marfan patients. This review examines current understan ding of the expression and assembly of fibrillin and describes new app roaches which are now being applied to elucidate the many outstanding structural, organisational and functional aspects of the fibrillin-con taining microfibrils.