Fibulins: physiological and disease perspectives

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 underst...

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Veröffentlicht in:	EMBO reports 2003-12, Vol.4 (12), p.1127-1131
Hauptverfasser:	Argraves, W Scott, Greene, Lisa M, Cooley, Marion A, Gallagher, William M
Format:	Artikel
Sprache:	eng
Schlagworte:	Amino Acid Sequence Animal models Animals Basement Membrane - metabolism Calcium-Binding Proteins - chemistry Calcium-Binding Proteins - genetics Calcium-Binding Proteins - physiology Cardiovascular System - metabolism Elastic Tissue - metabolism Extracellular Matrix Proteins - genetics Extracellular Matrix Proteins - metabolism Extracellular Matrix Proteins - physiology Fibers Gene Expression Regulation Humans Mice Mice, Knockout Models, Animal Molecular Sequence Data Mutation Neoplasms - genetics Neoplasms - metabolism Recombinant Proteins Review Wounds and Injuries - genetics Wounds and Injuries - metabolism
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Beschreibung
Zusammenfassung:	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.
ISSN:	1469-221X 1469-3178
DOI:	10.1038/sj.embor.7400033