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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 ...
F, Ramirez, L, Pereira
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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 ...
F, Ramirez, L, Pereira
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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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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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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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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, 2014Background 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 ...
M, Kira-Tatsuoka +4 more
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Fibrillin immunofluorescence in pseudoxanthoma elasticum
Journal of the American Academy of Dermatology, 1995Pseudoxanthoma 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 ...
M, Godfrey +6 more
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Fibrillin protein pleiotropy: Acromelic dysplasias
Matrix Biology, 2019The fibrillins are large extracellular matrix molecules that polymerize to form microfibrils. Fibrillin microfibrils are distinctive architectural elements that are both ubiquitous in the connective tissue space and also unique, displaying tissue-specific patterns.
Lynn Y, Sakai, Douglas R, Keene
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The fibrillin‐marfan syndrome connection
BioEssays, 1993AbstractA 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 ...
F, Ramirez, L, Pereira, H, Zhang, B, Lee
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Proteomic analysis of fibrillin‐rich microfibrils
PROTEOMICS, 2006Abstract MS has been used to investigate the composition of fibrillin‐rich microfibrils from non‐elastic and elastic tissues, and to compare fibrillin‐1 tryptic fingerprints derived from whole zonules, microfibrils and recombinant fibrillin‐1.
Cain, Stuart A. +5 more
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Finding fibrillin in cerebral artery dissection
Neurology, 2018Cerebral artery dissection (CeAD) is an important cause of stroke, particularly in the young. Identifying cerebral dissection benefits patients because it affects management, influences follow-up investigations, and may prompt evaluation for associated conditions such as connective tissue disease.1 Making the diagnosis of arterial dissection requires ...
Jickling G, LORENZANO S
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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.
Dirk Hubmacher, Dieter P. Reinhardt
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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.
Dirk Hubmacher, Dieter P. Reinhardt
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