Article

Fibrillin-containing microfibrils: structure and function in health and disease.

School of Biological Sciences, University of Manchester, U.K.
The International Journal of Biochemistry & Cell Biology (impact factor: 4.63). 09/1995; 27(8):747-60. pp.747-60
Source: PubMed

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.

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Keywords

connective tissue macromolecules
 
critical contribution
 
diverse extracellular matrices
 
Fibrillin-containing microfibrils
 
function relationships
 
functional aspects
 
heritable disorder
 
Marfan patients
 
outstanding structural
 
pleiotrophic connective tissue manifestations
 
primary structure
 
principal structural component fibrillin
 
recent elucidation
 
recent linkage
 

C M Kielty