Decreased elastin deposition and high proliferation of fibroblasts from Costello syndrome are related to functional deficiency in the 67-kD elastin-binding protein
about
Impaired elastic-fiber assembly by fibroblasts from patients with either Morquio B disease or infantile GM1-gangliosidosis is linked to deficiency in the 67-kD spliced variant of beta-galactosidase.Fibulin-4 regulates expression of the tropoelastin gene and consequent elastic-fibre formation by human fibroblastsNeuraminidase-1, a subunit of the cell surface elastin receptor, desialylates and functionally inactivates adjacent receptors interacting with the mitogenic growth factors PDGF-BB and IGF-2VE-statin/egfl7 regulates vascular elastogenesis by interacting with lysyl oxidasesLysosomal sialidase (neuraminidase-1) is targeted to the cell surface in a multiprotein complex that facilitates elastic fiber assemblyRetrovirally mediated overexpression of versican v3 reverses impaired elastogenesis and heightened proliferation exhibited by fibroblasts from Costello syndrome and Hurler disease patients.The hyperthermia-enhanced association between tropoelastin and its 67-kDa chaperone results in better deposition of elastic fibers.Fibulin-5/DANCE has an elastogenic organizer activity that is abrogated by proteolytic cleavage in vivoFurther delineation of cardiac abnormalities in Costello syndrome.Early-lethal Costello syndrome due to rare HRAS Tandem Base substitution (c.35_36GC>AA; p.G12E)-associated pulmonary vascular diseaseNeointima formed by arterial smooth muscle cells expressing versican variant V3 is resistant to lipid and macrophage accumulationEnhanced expression of CD44 variants in human atheroma and abdominal aortic aneurysm: possible role for a feedback loop in endothelial cells.Retinoblastoma protein modulates the inverse relationship between cellular proliferation and elastogenesisHuman iPS Cell-Derived Neurons Uncover the Impact of Increased Ras Signaling in Costello SyndromeAdvances in biomimetic regeneration of elastic matrix structures.Reduced elastogenesis: a clue to the arteriosclerosis and emphysematous changes in Schimke immuno-osseous dysplasia?Changes in elastin, elastin binding protein and versican in alveoli in chronic obstructive pulmonary diseaseCutaneous manifestations of Costello syndrome.Inhibition of versican expression by siRNA facilitates tropoelastin synthesis and elastic fiber formation by human SK-LMS-1 leiomyosarcoma smooth muscle cells in vitro and in vivo.Is SPARC an evolutionarily conserved collagen chaperone?Pro-inflammatory phenotype of COPD fibroblasts not compatible with repair in COPD lung.Connection between elastin haploinsufficiency and increased cell proliferation in patients with supravalvular aortic stenosis and Williams-Beuren syndromeCutaneous manifestations in Costello and cardiofaciocutaneous syndrome: report of 18 cases and literature review.Primary and secondary elastin-binding protein defect leads to impaired elastogenesis in fibroblasts from GM1-gangliosidosis patients.Deposition of insoluble elastin by pulmonary fibroblasts from patients with COPD is increased by treatment with versican siRNAOptimizing the molecular diagnosis of GALNS: novel methods to define and characterize Morquio-A syndrome-associated mutations.Elastic-fiber pathologies: primary defects in assembly-and secondary disorders in transport and deliveryC4ST-1/CHST11-controlled chondroitin sulfation interferes with oncogenic HRAS signaling in Costello syndrome.Lysyl Oxidase Induces Vascular Oxidative Stress and Contributes to Arterial Stiffness and Abnormal Elastin Structure in Hypertension: Role of p38MAPK.Elastin-derived peptides are new regulators of insulin resistance development in mice.Fluctuations of intracellular iron modulate elastin production.Anaesthetic implications of Costello syndrome.Myocardial storage of chondroitin sulfate-containing moieties in Costello syndrome patients with severe hypertrophic cardiomyopathy.Use of versican variant V3 and versican antisense expression to engineer cultured human skin containing increased content of insoluble elastin.Overgrowth with increased proliferation of fibroblast and matrix metalloproteinase activity related to reduced TIMP1: a newly recognized syndrome?
P2860
Q24538703-F9101C72-03EE-496F-8045-6A2159D4B1ABQ24634333-BE9D3718-1C35-4FDD-B5E1-CC60F307BF7BQ24647879-4C2AFE6E-CD74-4FD6-A2C5-94826A99930CQ24652188-DA5BA35C-8F04-4D8A-B7CB-F20878B3F2A7Q28284064-947EE667-F79E-48C1-A2A8-7357D047865AQ34286189-71512BE2-51E0-42D8-96E3-9E4CCC186010Q34401134-7FDD32DA-B740-4646-8D18-565427D7B6BDQ34610156-4E6CC0AF-62B8-4BEF-9AB1-AC7485547B75Q34811131-4E46F14B-53B3-4368-9A2C-27DD78C3C0A2Q34964138-8F863C6B-587E-47BA-B9A8-15959D58FF39Q35069763-F6634E57-07A4-495E-B296-C767126C0DABQ35103442-944AF8B0-9E23-41AB-ADB4-AD096315F0A1Q35378176-94FA2FEF-66CC-4D47-AC94-F2A55C7A45FFQ36431974-1171E833-E888-44C5-A9DB-5C76E6A12B59Q36552037-34309586-65EA-48CD-AF61-E13D0F46E2BBQ36599394-0283E8BE-6F56-46B0-AC55-40916858D1BAQ36678349-3C76437C-0440-4CC9-8AC9-BC1CF77621B9Q36703894-E3966F46-3636-4A5D-AC6E-9011B38EC68BQ36730629-D2239C6B-CD0D-4785-896F-3854523018CBQ36769633-B724D7D2-1C35-476B-8D3D-E94DED6DB354Q37295537-45BF006C-E26D-4834-BC68-E8592CB7D300Q37362266-411400D8-4C50-48A7-A02D-490D344B38BCQ38166789-1A1F6492-296F-4443-A9B4-BE60511898A0Q38318365-DABC4470-5161-4F06-B848-3D08319AD3E4Q38718718-67851553-738B-4F1E-8147-FEECF0B9FDADQ38924874-D465F7A6-561F-4793-802C-CF19644C2D5FQ39538723-C69893B2-4F60-4FB6-8A1F-34A5A2ADC659Q39654702-32D68968-892E-4C32-903A-44B436462EB1Q41466882-C401DEC1-21D3-4A1E-B64B-E375BA14B4E8Q42936597-6C6522A8-A8BF-4244-AD82-33845B0A3022Q45146750-1A81F23B-311F-42FF-9734-4C4916B2627EQ48633183-09A7946C-E516-4970-AE2F-EF43AB40FD35Q51934190-3F8941E1-7099-4CD7-9440-AA21BF9D3CDFQ53521965-A1A6B712-9F76-421D-A994-35D2377BEAB8Q56232655-92544797-6F74-4305-B734-AB14C4FC4468
P2860
Decreased elastin deposition and high proliferation of fibroblasts from Costello syndrome are related to functional deficiency in the 67-kD elastin-binding protein
description
2000 nî lūn-bûn
@nan
2000 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2000 թվականի մարտին հրատարակված գիտական հոդված
@hy
2000年の論文
@ja
2000年論文
@yue
2000年論文
@zh-hant
2000年論文
@zh-hk
2000年論文
@zh-mo
2000年論文
@zh-tw
2000年论文
@wuu
name
Decreased elastin deposition a ...... 67-kD elastin-binding protein
@ast
Decreased elastin deposition a ...... 67-kD elastin-binding protein
@en
Decreased elastin deposition a ...... 67-kD elastin-binding protein
@nl
type
label
Decreased elastin deposition a ...... 67-kD elastin-binding protein
@ast
Decreased elastin deposition a ...... 67-kD elastin-binding protein
@en
Decreased elastin deposition a ...... 67-kD elastin-binding protein
@nl
prefLabel
Decreased elastin deposition a ...... 67-kD elastin-binding protein
@ast
Decreased elastin deposition a ...... 67-kD elastin-binding protein
@en
Decreased elastin deposition a ...... 67-kD elastin-binding protein
@nl
P2093
P2860
P356
P1476
Decreased elastin deposition a ...... 67-kD elastin-binding protein
@en
P2093
P2860
P304
P356
10.1086/302829
P407
P50
P577
2000-03-01T00:00:00Z