Fibrillin: from domain structure to supramolecular assembly

In the last 5 years, significant progress has been made in understanding th e structure and function of all the major domains composing the fibrillins. A previous review [Meth. Enzymol. 245 (1994), 29] focused on the isolation of fibrillin monomers and fibrillin-containing polymers (microfibrils). In this article, information gained from recent studies which have further el ucidated molecular structure and investigated effects of mutations on struc tural and functional properties will be summarized. In addition, studies of functional domains in fibrillins which may be important in assembling micr ofibrils will be discussed. Throughout this review, the authors have attemp ted to identify areas of research which have been controversial. In the con clusion, we raise important questions which remain unresolved. (C) 2000 Els evier Science B.V./International Society of Matrix Biology. All rights rese rved.