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.

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.

Item
http://www.wikidata.org/entity/Q24538703
Fibulin-4 regulates expression of the tropoelastin gene and consequent elastic-fibre formation by human fibroblastsRetrovirally 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.GM1 gangliosidosis and Morquio B disease: an update on genetic alterations and clinical findings.Delineating the role of alterations in lipid metabolism to the pathogenesis of inherited skeletal and cardiac muscle disorders: Thematic Review Series: Genetics of Human Lipid Diseases.Reduced elastogenesis: a clue to the arteriosclerosis and emphysematous changes in Schimke immuno-osseous dysplasia?Latent TGF-β binding protein 4 promotes elastic fiber assembly by interacting with fibulin-5.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.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 injuryConnection between elastin haploinsufficiency and increased cell proliferation in patients with supravalvular aortic stenosis and Williams-Beuren syndromePrimary and secondary elastin-binding protein defect leads to impaired elastogenesis in fibroblasts from GM1-gangliosidosis patients.Elastic-fiber pathologies: primary defects in assembly-and secondary disorders in transport and deliveryCodependence of Bone Morphogenetic Protein Receptor 2 and Transforming Growth Factor-β in Elastic Fiber Assembly and Its Perturbation in Pulmonary Arterial Hypertension.Myocardial storage of chondroitin sulfate-containing moieties in Costello syndrome patients with severe hypertrophic cardiomyopathy.
P2860

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.

Item
http://www.wikidata.org/entity/Q24538703
description
2000 nî lūn-bûn
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2000 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
2000 թվականի հունիսին 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 elastic-fiber assembl ...... variant of beta-galactosidase
@nl
Impaired elastic-fiber assembl ...... variant of beta-galactosidase.
@ast
Impaired elastic-fiber assembl ...... variant of beta-galactosidase.
@en
type
label
Impaired elastic-fiber assembl ...... variant of beta-galactosidase
@nl
Impaired elastic-fiber assembl ...... variant of beta-galactosidase.
@ast
Impaired elastic-fiber assembl ...... variant of beta-galactosidase.
@en
prefLabel
Impaired elastic-fiber assembl ...... variant of beta-galactosidase
@nl
Impaired elastic-fiber assembl ...... variant of beta-galactosidase.
@ast
Impaired elastic-fiber assembl ...... variant of beta-galactosidase.
@en
P1433
P1476
P2093
P2860
P304
P31
P356
P407
P433
P478
P50
P577
P5875
P698
P921
P932
P356
P1433
P1476
Impaired elastic-fiber assembl ...... variant of beta-galactosidase
@en
P2093
P2860
P304
P31
P356
P407
P433
P478
P50
P577
P5875
P698
P921
P932