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 understanding of the normal and pathological roles of members of this formerly obscure family.
Argraves
W Scott
WS
Medical University of South Carolina, Department of Cell Biology, 173 Ashley Avenue, Charleston, South Carolina 29425, USA. argraves@musc.edu
Greene
Lisa M
LM
Cooley
Marion A
MA
Gallagher
William M
WM
D000595:Amino Acid Sequence; D000818:Animals; D001485:Basement Membrane / Q000378:metabolism; D002135:Calcium-Binding Proteins / Q000737:chemistry / Q000235:genetics / Q000502:physiology*; D002319:Cardiovascular System / Q000378:metabolism; D004547:Elastic Tissue / Q000378:metabolism; D016326:Extracellular Matrix Proteins / Q000235:genetics / Q000378:metabolism / Q000502:physiology; D005786:Gene Expression Regulation; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D023421:Models, Animal; D008969:Molecular Sequence Data; D009369:Neoplasms / Q000235:genetics / Q000378:metabolism; D011994:Recombinant Proteins; D014947:Wounds and Injuries / Q000235:genetics / Q000378:metabolism
D002135:Calcium-Binding Proteins; C403672:EFEMP2 protein, human; D016326:Extracellular Matrix Proteins; C492373:FBLN5 protein, human; C120769:Fbln5 protein, mouse; D011994:Recombinant Proteins; C060820:fibulin; C084612:fibulin 2