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The fibrillins

The International Journal of Biochemistry & Cell Biology, 1999
Fibrillins 1 and 2 are the main constituents of the extracellular microfibrils responsible for the biomechanical properties of most tissues and organs. They are cysteine-rich glycoproteins predominantly made of multiple repeats homologous to the calcium-binding epidermal growth factor module, and are translated as precursor proteins cleaved by furine ...
Lygia da Veiga Pereira   +1 more
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Fibrillins

2018
Fibrillins are one of the major components of supramolecular fibrous structures in the extracellular matrix of elastic and nonelastic tissues, termed microfibrils. Microfibrils provide tensile strength in nonelastic tissues and scaffolds for the assembly of tropoelastin in elastic tissues, and act a regulator of growth factor bioavailability and ...
Heena, Kumra, Dieter P, Reinhardt
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Fibrillin Microfibrils

2005
Fibrillin microfibrils are widely distributed extracellular matrix assemblies that endow elastic and nonelastic connective tissues with long-range elasticity. They direct tropoelastin deposition during elastic fibrillogenesis and form an outer mantle for mature elastic fibers.
Kielty, Cay M.   +3 more
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Immunohistochemical expression of fibrillin‐1 and fibrillin‐2 during tooth development

Journal of Periodontal Research, 2014
Background and ObjectiveOxytalan fibers are categorized as a microfibril assembly without elastin deposition, and are unique components in the periodontal ligament (PDL). However, little is known about their formation during PDL development. To clarify the mechanisms of oxytalan fiber formation in developing PDL, we performed immunohistochemical ...
Masao Ozaki   +4 more
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The structure and function of fibrillin.

Ciba Foundation symposium, 1995
Fibrillin is a very large molecule whose primary structure is now known from the cloning and sequencing of 10 kb of cDNA. Immunohistochemical results suggest that one of the functions of fibrillin molecules is to contribute to the structure of the microfibril.
Lynn Y. Sakai   +2 more
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Fibrillin immunofluorescence in pseudoxanthoma elasticum

Journal of the American Academy of Dermatology, 1995
Pseudoxanthoma elasticum (PXE) is a rare heritable connective tissue disorder manifested by skin, ocular, and cardiovascular anomalies. The basic defect is unknown; however, the microscopic findings are indicative of defects in elastic fibers. Among the components of the elastic fibers are elastin and elastin-associated microfibrils.We assessed the ...
Jason Cisler   +6 more
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The fibrillin‐marfan syndrome connection

BioEssays, 1993
AbstractA few years ago no one would have suspected that the well‐known disorder of connective tissue, Marfan syndrome, could be caused by mutations in a recently discovered extracellular component, fibrillin. Likewise, nobody would have predicted that fibrillin represents a small family of proteins that are associated with several pheno‐typically ...
Brendan Lee   +3 more
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Microfibrils and Fibrillin

2010
Microfibrils are supramolecular structures ubiquitously found in the extracellular matrix of elastic and nonelastic tissues. The three members of the cysteine-rich fibrillin family constitute the core of microfibrils. Mutations in fibrillin-1 and -2 lead to a number of heritable connective tissue disorders termed fibrillinopathies.
Dieter P. Reinhardt, Dirk Hubmacher
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