Impaired elastogenesis in Hurler disease: dermatan sulfate accumulation linked to deficiency in elastin-binding protein and elastic fiber assembly.
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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.Neuraminidase-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-2Atypical microbial infections of digestive tract may contribute to diarrhea in mucopolysaccharidosis patients: a MPS I case studyLysosomal sialidase (neuraminidase-1) is targeted to the cell surface in a multiprotein complex that facilitates elastic fiber assemblyGender-related dimorphism in aortic insufficiency in murine mucopolysaccharidosis type IThe factors affecting lipid profile in adult patients with MucopolysaccharidosisUpregulation of elastase activity in aorta in mucopolysaccharidosis I and VII dogs may be due to increased cytokine expression.Three novel beta-galactosidase gene mutations in Han Chinese patients with GM1 gangliosidosis are correlated with disease severity.Impact of pre-existing elastic matrix on TGFβ1 and HA oligomer-induced regenerative elastin repair by rat aortic smooth muscle cellsRetrovirally mediated overexpression of versican v3 reverses impaired elastogenesis and heightened proliferation exhibited by fibroblasts from Costello syndrome and Hurler disease patients.Management of mucopolysaccharidosis type IH (Hurler's syndrome) presenting in infancy with severe dilated cardiomyopathy: a single institution's experience.The hyperthermia-enhanced association between tropoelastin and its 67-kDa chaperone results in better deposition of elastic fibers.Arterial pathology in canine mucopolysaccharidosis-I and response to therapyRetinoblastoma protein modulates the inverse relationship between cellular proliferation and elastogenesisUltrasonographic Features of Hip Joints in Mucopolysaccharidoses Type I and II.Cardiac disease in patients with mucopolysaccharidosis: presentation, diagnosis and managementUncoupling of Elastin Complex Receptor during In Vitro Aging Is Related to Modifications in Its Intrinsic Sialidase Activity and the Subsequent Lactosylceramide Production.Pathogenesis of aortic dilatation in mucopolysaccharidosis VII mice may involve complement activation.Adverse Effects of Genistein in a Mucopolysaccharidosis Type I Mouse ModelAdvances 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 diseaseInhibition 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.Endothelial function in children and adolescents with mucopolysaccharidosis.The role of caveolin-1 in pulmonary matrix remodeling and mechanical properties.The mucopolysaccharidoses: a success of molecular medicine.Retrovirally mediated overexpression of glycosaminoglycan-deficient biglycan in arterial smooth muscle cells induces tropoelastin synthesis and elastic fiber formation in vitro and in neointimae after vascular injuryUpregulation of elastase proteins results in aortic dilatation in mucopolysaccharidosis I mice.Clinical characterization of cardiovascular abnormalities associated with feline mucopolysaccharidosis I and VIIsolation and Quantification of Glycosaminoglycans from Human Hair ShaftConnection between elastin haploinsufficiency and increased cell proliferation in patients with supravalvular aortic stenosis and Williams-Beuren syndromeThe effect of recombinant human iduronate-2-sulfatase (Idursulfase) on growth in young patients with mucopolysaccharidosis type II.The bladder extracellular matrix. Part I: architecture, development and disease.Efficacy of recombinant human alpha-L-iduronidase (laronidase) on restricted range of motion of upper extremities in mucopolysaccharidosis type I patients.Review of molecular and mechanical interactions in the aortic valve and aorta: implications for the shared pathogenesis of aortic valve disease and aortopathy.Primary and secondary elastin-binding protein defect leads to impaired elastogenesis in fibroblasts from GM1-gangliosidosis patients.Mechanistic and therapeutic overview of glycosaminoglycans: the unsung heroes of biomolecular signaling.Optimizing 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 deliveryGlycosaminoglycan metabolism defects and atherosclerosis: frequent association of endothelial dysfunction in patients with Mucopolysaccharidosis.
P2860
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P2860
Impaired elastogenesis in Hurler disease: dermatan sulfate accumulation linked to deficiency in elastin-binding protein and elastic fiber assembly.
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
Impaired elastogenesis in Hurl ...... in and elastic fiber assembly.
@ast
Impaired elastogenesis in Hurl ...... in and elastic fiber assembly.
@en
type
label
Impaired elastogenesis in Hurl ...... in and elastic fiber assembly.
@ast
Impaired elastogenesis in Hurl ...... in and elastic fiber assembly.
@en
prefLabel
Impaired elastogenesis in Hurl ...... in and elastic fiber assembly.
@ast
Impaired elastogenesis in Hurl ...... in and elastic fiber assembly.
@en
P2860
P1476
Impaired elastogenesis in Hurl ...... in and elastic fiber assembly.
@en
P2093
P2860
P304
P356
10.1016/S0002-9440(10)64961-9
P407
P577
2000-03-01T00:00:00Z