Purification and partial characterization of fibrillin, a cysteine-rich structural component of connective tissue microfibrils
about
Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndromeLatent TGF-β binding protein-2 is essential for the development of ciliary zonule microfibrilsStructure of the integrin binding fragment from fibrillin-1 gives new insights into microfibril organizationStructure and expression of fibrillin-2, a novel microfibrillar component preferentially located in elastic matricesFunctional domains on elastin and microfibril-associated glycoprotein involved in elastic fibre assemblyLosartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndromeThe molecular genetics of Marfan syndrome and related disordersThe molecular genetics of Marfan syndrome and related microfibrillopathiesLatent transforming growth factor-beta binding protein domains involved in activation and transglutaminase-dependent cross-linking of latent transforming growth factor-betaAbsence of autoantibodies against correctly folded recombinant fibrillin-1 protein in systemic sclerosis patientsStructure of the Fibrillin-1 N-Terminal Domains Suggests that Heparan Sulfate Regulates the Early Stages of Microfibril AssemblyDiagnostic Exome Sequencing Identifies a Novel Gene, EMILIN1, Associated with Autosomal-Dominant Hereditary Connective Tissue DiseaseDevelopmental expression of fibrillin genes suggests heterogeneity of extracellular microfibrilsADAMTSL-6 is a novel extracellular matrix protein that binds to fibrillin-1 and promotes fibrillin-1 fibril formationExtracellular fibrillar structure of latent TGF beta binding protein-1: role in TGF beta-dependent endothelial-mesenchymal transformation during endocardial cushion tissue formation in mouse embryonic heartEmilin, a component of elastic fibers preferentially located at the elastin-microfibrils interfaceIntracellular mechanisms of molecular recognition and sorting for transport of large extracellular matrix moleculesEffects of proline cis-trans isomerization on TB domain secondary structure.Rationale and design of a randomized clinical trial of beta-blocker therapy (atenolol) versus angiotensin II receptor blocker therapy (losartan) in individuals with Marfan syndromeBiogenesis and function of fibrillin assemblies.Marfan syndrome.Microfibril structure masks fibrillin-2 in postnatal tissuesFibrillins in adult human ovary and polycystic ovary syndrome: is fibrillin-3 affected in PCOS?Expression of a mutant human fibrillin allele upon a normal human or murine genetic background recapitulates a Marfan cellular phenotype.An extra cysteine in one of the non-calcium-binding epidermal growth factor-like motifs of the FBN1 polypeptide is connected to a novel variant of Marfan syndrome.Quantitative differences in biosynthesis and extracellular deposition of fibrillin in cultured fibroblasts distinguish five groups of Marfan syndrome patients and suggest distinct pathogenetic mechanisms.Marfan phenotype variability in a family segregating a missense mutation in the epidermal growth factor-like motif of the fibrillin gene.Fifteen novel FBN1 mutations causing Marfan syndrome detected by heteroduplex analysis of genomic ampliconsIdentifying specific proteins involved in eggshell membrane formation using gene expression analysis and bioinformatics.A compound-heterozygous Marfan patient: two defective fibrillin alleles result in a lethal phenotype.A novel FBN1 mutation in a Chinese family with isolated ectopia lentis.Identification of a novel FBN1 gene mutation in a large Pakistani family with Marfan syndrome.The Tight skin mouse: demonstration of mutant fibrillin-1 production and assembly into abnormal microfibrils.New insights into the assembly of extracellular microfibrils from the analysis of the fibrillin 1 mutation in the tight skin mouse.C677T polymorphism in the methylenetetrahydrofolate reductase gene is associated with primary closed angle glaucoma.Bovine latent transforming growth factor beta 1-binding protein 2: molecular cloning, identification of tissue isoforms, and immunolocalization to elastin-associated microfibrilsBiogenesis of extracellular microfibrils: Multimerization of the fibrillin-1 C terminus into bead-like structures enables self-assemblyExtracellular matrix in the trabecular meshwork.Fibrillin assembly requires fibronectin.Two mutations in Marfan syndrome resulting in truncated fibrillin polypeptides.
P2860
Q22065344-91D89F2E-E3CC-4A1B-854A-08151821AE82Q24299050-4E13FC23-8FA1-49D5-AD7F-8B26C49CF04DQ24323342-9E49BCC6-223D-4462-A747-BD572825A01CQ24336069-5FE71AF0-1FDA-4587-9C94-05718CAD937EQ24529881-6714FA5C-BAF8-445E-882B-F1B1D15E024BQ24548457-C33F1521-8574-4B3E-8A40-E3DA24AA82C5Q24656172-1B2F8D8B-A3CA-44A3-B3F0-ED0C166CE2F9Q24681593-26841F9E-8B17-4579-BAF7-E20A8BA8112FQ24683923-B96C1878-9279-414B-A449-F7477E308331Q24812752-5C69E6F6-7E19-4382-B987-CDAED21C9422Q27679940-E4ACBB2E-A021-42CB-A6E7-5F8296001C60Q28118637-45DA4105-AF2B-4D0A-8C4E-5238C0A1F527Q28504747-D5BB9D4F-01F4-4E6D-92E5-9B48B87118E6Q28508725-378A628D-EA0B-40FA-97BF-AA717E0A90C0Q28511184-D9B8E372-DC34-480E-A123-D426E0736568Q28610088-2BD0C2D4-1557-4949-8D75-D500DD6E7D10Q30008858-E68CE808-825A-4663-B8D4-D163AC0FEE0DQ30431820-D99CB362-F623-47BF-A821-C3423C0402EDQ30476899-B3C91B83-416D-4D04-9882-B56197E8B655Q33641386-DC9C7767-1C47-45F7-8701-FECDE62ABF4BQ33677345-03FFC4CD-1E6A-48B5-9BFB-6B7915E282F1Q33924430-C5193C0E-8D1A-465E-8FE1-449620525FAEQ34141743-5B1A5B49-8304-4C98-914E-AD9646BF1D99Q34203531-D788AD52-E9DD-4D7C-AE86-6B5484DD92AAQ34229237-00D4D733-1530-49FE-ABC9-80D0F463B7C1Q34234444-53348783-A31D-49EF-B0BB-1C3D07951645Q35602882-C5994FA8-8DC9-4D6A-AB23-C8085F388FE0Q35643503-3D2EE380-9B29-4D2D-813C-6891E95389A6Q35809137-EBA6D2B8-05F1-49C4-A3A6-1EE933BB5FB5Q35889756-F0A1895E-E4EB-42B2-B639-1E5FCF56EF9CQ35910866-EA21E85E-D012-44DF-A5ED-766BC107716BQ36146050-BD9800C0-E403-4E52-9812-415B5F61877EQ36255202-A60258E1-214F-4391-BC7B-96978CA67B6AQ36328488-9788ADCF-51D6-4577-AD17-5D44F6212E6DQ36514415-60611C57-4162-4D14-9A6D-B11C1D4956DFQ36556405-D74D2F3A-4715-48E4-9AD9-3CF2302DADACQ36638779-ED7EC773-DDC9-46DB-A0CC-EE0168EAAF5EQ36646381-07C580DB-6230-4B03-9BC3-5809B59FF78AQ37078574-5CD4AF68-91DE-4AC9-AEF2-C9999E8C1F1AQ37084846-E5E8D731-C3D8-49FA-AC6D-D702688D68BA
P2860
Purification and partial characterization of fibrillin, a cysteine-rich structural component of connective tissue microfibrils
description
1991 nî lūn-bûn
@nan
1991 թուականի Օգոստոսին հրատարակուած գիտական յօդուած
@hyw
1991 թվականի օգոստոսին հրատարակված գիտական հոդված
@hy
1991年の論文
@ja
1991年論文
@yue
1991年論文
@zh-hant
1991年論文
@zh-hk
1991年論文
@zh-mo
1991年論文
@zh-tw
1991年论文
@wuu
name
Purification and partial chara ...... connective tissue microfibrils
@ast
Purification and partial chara ...... connective tissue microfibrils
@en
Purification and partial chara ...... connective tissue microfibrils
@en-gb
Purification and partial chara ...... connective tissue microfibrils
@nl
type
label
Purification and partial chara ...... connective tissue microfibrils
@ast
Purification and partial chara ...... connective tissue microfibrils
@en
Purification and partial chara ...... connective tissue microfibrils
@en-gb
Purification and partial chara ...... connective tissue microfibrils
@nl
prefLabel
Purification and partial chara ...... connective tissue microfibrils
@ast
Purification and partial chara ...... connective tissue microfibrils
@en
Purification and partial chara ...... connective tissue microfibrils
@en-gb
Purification and partial chara ...... connective tissue microfibrils
@nl
P2093
P1476
Purification and partial chara ...... connective tissue microfibrils
@en
P2093
H P Bächinger
R W Glanville
P304
P407
P577
1991-08-05T00:00:00Z