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Topic: Fibrillin

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Connective tissue

Marfan syndrome

	Fibrillin - Wikipedia, the free encyclopedia
		Fibrillin is a glycoprotein, which is essential for the formation of elastic fibres found in connective tissue.
		Fibrillin-1 is a major component of the microfibrils that form a sheath surrounding the amorphous elastin.
		It is believed that the microfibrils are composed of end-to-end polymers of fibrillin.
	en.wikipedia.org /wiki/Fibrillin (189 words)

	Fbn2 - fibrillin 2
		Of mice and Marfan: genetic linkage analyses of the fibrillin genes, Fbn1 and Fbn2, in the mouse genome.
		Mutations of the mouse Twist and sy (fibrillin 2) genes induced by chemical mutagenesis of ES cells.
		Fbn2 is a homologue of FBN2 (fibrillin 2 (congenital contractural arachnodactyly)) from Homo sapiens.
	www.ihop-net.org /UniPub/iHOP/gg/120959.html (642 words)

	New Insights into the Assembly of Extracellular Microfibrils from the Analysis of the Fibrillin 1 Mutation in the Tight ...
		Schematic illustration of the wild-type (WT) and mutant (Tsk) fibrillin 1 proteins with the wild-type and mutant sequences at the NH -terminal junction of the Tsk duplication (gray segment).
		of Tsk and wild-type fibrillin 1 in Tsk/+ mice decreases the
		with quantitation of fibrillin 1 expression in Fig 4 a, the
	www.jcb.org /cgi/content/full/150/3/667 (5755 words)

	[No title]
		The two fibrillin genes are differentially expressed in terms of developmental stage and tissue distribution, and probably fulfill similar but distinct roles, with fibrillin-1 providing mainly force-bearing structural support and fibrillin-2 regulating the process of elastic fiber assembly.
		Proper formation of fibrillin aggregates is likely to depend on the proper conformation of participating fibrillin monomers, such that mutant monomers may interfere with the mechanisms needed for polymerization.
		Fibrillin is difficult to express as a full-length molecule with present technology, and most in vitro experiments are performed on recombinant fibrillin-1 fragments corresponding to smaller or larger segments of fibrillin-1.
	www.charite.de /ch/medgen/eumedis/medgen05/marfan.html (3587 words)

	Isolation of rat fibrillin-1 cDNA and its relevance in metanephric development -- Kanwar et al. 275 (5): 710 -- AJP - ...
		Fibrillin binds calcium and is coded by cDNAs that reveal a multidomain structure and alternatively spliced exons at the 5' end.
		Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene.
		Genetic linkage of the Marfan syndrome, ectopia lentis, and congenital contractural arachnodactyly to the fibrillin genes on chromosomes 15 and 5.
	ajprenal.physiology.org /cgi/content/full/275/5/F710 (6450 words)

	Marfan syndrome - Wikipedia, the free encyclopedia
		Marfan syndrome is associated with incomplete penetrance, therefore not all persons carrying the mutation develop the disease.
		Without the structural support provided by fibrillin, many connective tissues are weakened, which can have severe consequences on support and stability.
		A related disease has been found in mice, and the study of mouse fibrillin synthesis and secretion, and connective tissue formation, has begun to further our understanding of Marfan syndrome in humans.
	en.wikipedia.org /wiki/Marfan_syndrome (866 words)

	Homo- and Heterotypic Fibrillin-1 and -2 Interactions Constitute the Basis for the Assembly of Microfibrils -- Lin et ...
		The C-terminal fragments of both fibrillins have been used as soluble ligands and the N-terminal fragments as immobilized ligands.
		Fibrillin fragments have been incubated for 10 min at the temperatures indicated, and then used in homo- and heterotypic fibrillin solid phase interaction assays.
		are responsible for mediating the fibrillin homo- and heterotypic
	www.jbc.org /cgi/content/full/277/52/50795 (6031 words)

	The Supramolecular Organization of Fibrillin-rich Microfibrils -- Baldock et al. 152 (5): 1045 -- The Journal of Cell ...
		is emphasized by linkage of fibrillin mutations to Marfan syndrome
		Schematic diagram depicting the folding of fibrillin molecules in a beaded microfibril.
		Sakai, L.Y., Keene, D.R., and Engvall, E. Fibrillin, a new 350-kd glycoprotein, is a component of extracellular microfibrils.
	www.jcb.org /cgi/content/full/152/5/1045 (5768 words)

	The Microfibrillar Proteins MAGP-1 and Fibrillin-1 Form a Ternary Complex with the Chondroitin Sulfate Proteoglycan ...
		MAGP-1 and Fibrillin Coprecipitate in the Absence of CSPGs
		MAGP-1 and fibrillin coprecipitate in the absence of decorin.
		Kielty, C.M., Whittaker, S.P., and Shuttleworth, C.A. Fibrillin: evidence that chondroitin sulfate proteoglycans are components of microfibrils and associate with newly synthesized monomers.
	www.molbiolcell.org /cgi/content/full/11/5/1499 (5175 words)

	Abnormal Extracellular Matrix Protein Transport Associated With Increased Apoptosis of Vascular Smooth Muscle Cells in ...
		caused by a mutation in the fibrillin gene FBN1.
		Fibrillin, fibronectin, and tenascin expression in cultured control vascular smooth muscle cells, Marfan syndrome (MS) and bicuspid aortic valve (BAV) using Western blot and quantitative densitometry (mean +/- SD, arbitrary units) For each protein, there was no significant difference (P>0.05) between the MS or BAV groups and the control group.
		Immunohistochemical abnormalities of fibrillin in cardiovascular tissues in Marfan’s syndrome.
	circ.ahajournals.org /cgi/content/full/108/10_suppl_1/II-329 (3095 words)

	Substrate-Dependent Morphology of Supramolecular Assemblies: Fibrillin and Type-VI Collagen Microfibrils -- Sherratt et ...
		Fibrillin microfibrils (FIB) and type-VI collagen microfibrils (VI) extracted from the same tissue sample were identified on all substrates.
		Fibrillin: evidence that chondroitin sulphate proteoglycans are components of microfibrils and associate with newly synthesised monomers.
		Fibrillin microfibrils are stiff reinforcing fibres in compliant tissues.
	www.biophysj.org /cgi/content/full/86/5/3211 (5570 words)

	Gene Expression and Accumulation of Fibrillin-1, Fibrillin-2, and Tropoelastin in Cultured Periodontal Fibroblasts -- ...
		Fibrillin, a new 350-kD glycoprotein, is a component of extracellular microfibrils.
		Interaction of tropoelastin with the amino-terminal domains of fibrillin-1 and fibrillin-2 suggests a role for the fibrillins in elastic fiber assembly.
		Expression of fibrillins and tropoelastin by human gingival and periodontal ligament fibroblasts in vitro.
	jdr.iadrjournals.org /cgi/content/full/81/11/771 (2788 words)

	Godfrey
		Schaefer, G.B. and Godfrey, M. Quantitation of fibrillin immunofluorescence in fibroblast cultures in the Marfan syndrome.
		These studies include direct analysis of patient material for diagnostic purposes, the design of mouse models using transgenic or knockout technologies, and the identification of chick fibrillin homologs for studies of the ontogeny of the cardiovascular system and limb development.
		Fibrillin immunochemical analyses in the Marfan syndrome and related disorders.
	www.unmc.edu /UNCOM/labinfo/Godfrey.htm (868 words)

	Muscle fibrillin deficiency in Marfan's syndrome myopathy -- Behan et al. 74 (5): 633 -- Journal of Neurology, ...
		abnormal fibrillin immunoreactivity in the endomysium and perimysium.
		It can be seen that, compared with the control, fibrillin appears defective, wispy, and fragmented in the three cases.
		Marfan syndrome caused by a recurrent de novo nonsense mutation in the fibrillin gene.
	jnnp.bmjjournals.com /cgi/content/full/74/5/633 (3101 words)

	Lung Elastin and Matrix -- Starcher 117 (5 Supplement 1): 229 -- Chest
		the fibrillin gene are responsible for Marfan’s syndrome.
		Saki, LY, Keene, DR, Engvall, E (1986) Fibrillin, a new 350-kD glycoprotein, is a component of extracellular microfibrils.
		Kattunen, L, Kattunen, L, Puhakka, L, et al (1994) Mutations in the fibrillin gene responsible for dominant ectopia lentis and neonatal Marfan syndrome.
	www.chestjournal.org /cgi/content/full/117/5_suppl_1/229S-a (3172 words)

	Fibrillin-1 in Human Cartilage: Developmental Expression and Formation of Special Banded Fibers -- Keene et al. 45 (8): ...
		The major domains of recombinant fibrillin peptides and the position of the peptides within fibrillin-1 and fibrillin-2 is illustrated.
		Confocal immunofluorescence of the same 14-year-old rib used in Figure 10A-C. Using MAb 69, fibrillin was localized to the periphery of a human chondrocyte.
		Corson GM, Chalberg SC, Dietz HC, Charbonneau NL, Sakai LY (1993) Fibrillin binds calcium and is coded by cDNAs that reveal a multidomain structure and alternatively spliced exons at the 5' end.
	www.jhc.org /cgi/content/full/45/8/1069 (5059 words)

	NEJM -- Genetic linkage of the Marfan syndrome, ectopia lentis, and congenital contractural arachnodactyly to the ...
		NEJM -- Genetic linkage of the Marfan syndrome, ectopia lentis, and congenital contractural arachnodactyly to the fibrillin genes on chromosomes 15 and 5.
		Using specific markers for the fibrillin genes, we performed genetic linkage analysis in 28 families with the Marfan syndrome and 8 families with four phenotypically related disorders--congenital contractural arachnodactyly (3 families), ectopia lentis (2), mitral-valve prolapse syndrome (2), and annuloaortic ectasia (1).
		The Marfan syndrome appears to be caused by mutations in a single fibrillin gene on chromosome 15.
	content.nejm.org /cgi/content/abstract/326/14/905 (919 words)

	Energy Citations Database (ECD) - Energy and Energy-Related Bibliographic Citations
		To determine the sensitivity and specificity of fibrillin protein abnormalities in the diagnosis of MFS, we studied dermal fibroblasts from 57 patients with classical MFS, 15 with equivocal MFS, 8 with single-organ manifestations, and 16 with other connective tissue disorders including homocystinuria and Ehlers-Danlos syndrome.
		Abnormal fibrillin metabolism was identified in 70 samples that were classified into four different groups based on quantitation of fibrillin synthesis and matrix deposition.
		Our results indicate that fibrillin defects at the protein level per se are not specific for MFS, but that the drastically reduced fibrillin deposition, caused by a dominant-negative effect of abnormal fibrillin molecules in individuals defined as groups II and IV, is of prognostic and possibly diagnostic significance.
	www.osti.gov /energycitations/product.biblio.jsp?osti_id=466720 (346 words)

	eMedicine - Marfan Syndrome : Article by Harold Chen, MD, MS, FAAP, FACMG (Site not responding. Last check: 2007-10-23)
		The disorder results from molecular defects in the fibrillin gene that are responsible for the impaired structural integrity of the skeletal, ocular, and cardiovascular systems.
		The gene encodes the glycoprotein fibrillin, a major building block of microfibrils, which constitute the structural components of the suspensory ligament of the lens and which serve as substrates for elastin in the aorta and other connective tissues.
		Three other proposed pathophysiologic mechanisms are a disturbance of tissue homeostasis of elastic fibers, increased susceptibility of fibrillin to proteolysis, and marked dysregulation of transforming growth factor-beta (TGF-beta) activation and signaling resulting in apoptosis, as shown in the developing lung of mice deficient in fibrillin-1.
	www.emedicine.com /PED/topic1372.htm (5787 words)

	Fibrillin antibodies from RDI Divison of Fitzgerald Industries Intl (Site not responding. Last check: 2007-10-23)
		Fibrillin-1 is an extracellular acidic protein with a high cysteine content and an extended thread-like shape with mosaic composition of different types of extracellular modules.
		Most of the fibrillin molecule is contributed by 47 epidermal growth factor-like (EGF-like) repeats; 43 of them have a consensus sequence for calcium binding (cb).
		The fibrillins are found throughout the connective tissue as integral components of extended fibrils.
	www.researchd.com /rdiabs/fibrillin.htm (364 words)

	Latent Transforming Growth Factor beta -binding Protein 1 Interacts with Fibrillin and Is a Microfibril-associated ...
		Binding Studies-- Interactions between LTBP and fibrillin were investigated by solid phase ELISA binding or blot overlay assays.
		LTBP-1 and fibrillin was prominent in tendon, perichondrium, cartilage,
		LTBP-1 Binds to Fibrillins-- Blot overlay assays were utilized to screen for regions of fibrillins that might interact with LTBP-1.
	www.jbc.org /cgi/content/full/278/4/2750 (5621 words)

	Fibrillin and other matrix proteins in mitral valve prolapse syndrome -- Nasuti et al. 77 (2): 532 -- The Annals of ...
		Fibrillin and other matrix proteins in mitral valve prolapse syndrome -- Nasuti et al.
		Fibrillin and other matrix proteins in mitral valve prolapse syndrome
		diffuse, weaker, and nonlaminar pattern of staining for fibrillin.
	ats.ctsnetjournals.org /cgi/content/abstract/77/2/532 (369 words)

	Transglutaminase Activity in the Eye: Cross-linking in Epithelia and Connective Tissue Structures -- Raghunath et al. ...
		fibrillin was present only in the walls of retinal vessels (Fig.
		Fibrillin in the optic nerve was localized to capillaries
		Qian, R-Q, Glanville, RW (1997) The alignment of fibrillin molecules in elastic microfibrils is defined by TGase derived cross-link Biochemistr 36,15841-15847
	www.iovs.org /cgi/content/full/40/12/2780 (4639 words)

	SICKLE CELL ANEMIA: CHROMOSOME AND INHERITANCE
		Fibrillin-1 gene instructs our cells to produce the protein fibrillin for the making of connective tissues in the human body system.
		“Most researchers say that Marfan syndrome is caused by a mutation in the gene for fibrillin (specifically the fibrillin-1, or FBN-1 gene).
		Fibrillin: Does it really cause Marfan syndrome?, www.ctds.info/fibrillin.html
	fog.ccsf.cc.ca.us /~cpogge/disease/Steven/Marfan/inheritance.html (561 words)

	The molecular genetics of Marfan syndrome and related microfibrillopathies -- Robinson and Godfrey 37 (1): 9 -- Journal ...
		Consensus sequences were determined from sequences taken from the references cited in fig 1.
		The fibrillins are structurally related to the LTBP gene family.
		The fact that individual fibrillin monomers possess a width of 2.2 nm and the microfibrils a width of 10-12 nm
	jmg.bmjjournals.com /cgi/content/full/37/1/9 (6341 words)

	UniProtKB/Swiss-Prot entry P35555 [FBN1_HUMAN] Fibrillin-1 (Site not responding. Last check: 2007-10-23)
		Hewett D.R. Lynch J.R. Child A. Sykes B.C. "A new missense mutation of fibrillin in a patient with Marfan syndrome.";
		Kainulainen K. Karttunen L. Puhakka L. Sakai L. Peltonen L. "Mutations in the fibrillin gene responsible for dominant ectopia lentis and neonatal Marfan syndrome.";
		: Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles.
	www.expasy.org /uniprot/P35555 (2229 words)

	Marfan Syndrome: Can Alternative Treatment with Diet Help?
		Most researchers say that Marfan syndrome (MFS) is caused by a mutation in the gene for fibrillin (specifically the fibrillin-1, or FBN1 gene).
		However, recent studies show that most people with Marfan syndrome (72%) have not been found to have fibrillin mutations.
		Click here to read my section on interesting fibrillin research and Marfan syndrome.
	www.ctds.info /marfan.html (1161 words)

	Ultrastructural morphology and expression of proteoglycans, {beta}ig-h3, tenascin-C, fibrillin-1, and fibronectin in ...
		the FBN1 gene that encodes fibrillin is responsible for Marfan
		In an earlier study of BK, fibrillin-1 was expressed in the epithelial basement membrane, the stroma, DM, the area of subepithelial
		Immunolocalization of fibrillin and type VII collagen in keratoconus corneas.
	bjo.bmjjournals.com /cgi/content/full/85/6/720 (4489 words)

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