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Fibulins: physiological and disease perspectives
W Scott Argraves1, Lisa M Greene, Marion A Cooley
1Medical University of South Carolina, Department of Cell Biology, 173 Ashley Avenue, Charleston, South Carolina 29425, USA. argraves@musc.edu
Abstract:
The fibulins are a family of proteins that are associated with basement membranes and elastic extracellular matrix fibres. This review summarizes findings from studies of animal models of fibulin deficiency, human fibulin gene mutations, human tumours and injury models that have advanced our understanding of the normal and pathological roles of members of this formerly obscure family.
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