The 67-kDa enzymatically inactive alternatively spliced variant of beta-galactosidase is identical to the elastin/laminin-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.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-2Fragments of extracellular matrix as mediators of inflammationThe Elastin Receptor Complex: A Unique Matricellular Receptor with High Anti-tumoral PotentialSerine carboxypeptidase SCPEP1 and Cathepsin A play complementary roles in regulation of vasoconstriction via inactivation of endothelin-1Crystal Structure of Human -Galactosidase: STRUCTURAL BASIS OF GM1 GANGLIOSIDOSIS AND MORQUIO B DISEASESLysosomal sialidase (neuraminidase-1) is targeted to the cell surface in a multiprotein complex that facilitates elastic fiber assemblyThe elastin receptor complex transduces signals through the catalytic activity of its Neu-1 subunitDecreased elastin deposition and high proliferation of fibroblasts from Costello syndrome are related to functional deficiency in the 67-kD elastin-binding proteinAlternative splice variants in TIM barrel proteins from human genome correlate with the structural and evolutionary modularity of this versatile protein foldCharacterising alternate splicing and tissue specific expression in the chicken from ESTsSR proteins and the nonsense-mediated decay mechanism are involved in human GLB1 gene alternative splicing.Elevated concentrations of matrix metalloproteinase-12 and elastin degradation products in the sera of pregnant women infected with Toxoplasma gondiiImpaired elastogenesis in Hurler disease: dermatan sulfate accumulation linked to deficiency in elastin-binding protein and elastic fiber assembly.Multi-system disorders of glycosphingolipid and ganglioside metabolismGlycosylation modulates melanoma cell α2β1 and α3β1 integrin interactions with type IV collagenMolecular consequences of the pathogenic mutation in feline GM1 gangliosidosisThree novel beta-galactosidase gene mutations in Han Chinese patients with GM1 gangliosidosis are correlated with disease severity.Retrovirally mediated overexpression of versican v3 reverses impaired elastogenesis and heightened proliferation exhibited by fibroblasts from Costello syndrome and Hurler disease patients.Elastin fragments drive disease progression in a murine model of emphysemaThe hyperthermia-enhanced association between tropoelastin and its 67-kDa chaperone results in better deposition of elastic fibers.Constant splice-isoform ratios in human lymphoblastoid cells support the concept of a splico-statGlycosaminoglycan storage disorders: a reviewGM1 gangliosidosis and Morquio B disease: an update on genetic alterations and clinical findings.Uncoupling of Elastin Complex Receptor during In Vitro Aging Is Related to Modifications in Its Intrinsic Sialidase Activity and the Subsequent Lactosylceramide Production.Interaction between the elastin peptide VGVAPG and human elastin binding proteinChanges in elastin, elastin binding protein and versican in alveoli in chronic obstructive pulmonary diseaseApplying elastic fibre biology in vascular tissue engineering.Elastin-Derived Peptides Promote Abdominal Aortic Aneurysm Formation by Modulating M1/M2 Macrophage Polarization.Mechanisms and treatment of cardiovascular disease in Williams-Beuren syndrome.Attacking the multi-tiered proteolytic pathology of COPD: new insights from basic and translational studies.Connection between elastin haploinsufficiency and increased cell proliferation in patients with supravalvular aortic stenosis and Williams-Beuren syndromeMatrix elastin: a promising biomarker for chronic obstructive pulmonary disease.Primary and secondary elastin-binding protein defect leads to impaired elastogenesis in fibroblasts from GM1-gangliosidosis patients.Molecular responses of choroidal endothelial cells to elastin derived peptides through the elastin-binding protein (GLB1).Elastic-fiber pathologies: primary defects in assembly-and secondary disorders in transport and deliveryAstrocytoma cell interaction with elastin substrates: implications for astrocytoma invasive potential.Partial amino acid sequence and mRNA analysis of cytosolic pyridoxine-beta-D-glucoside hydrolase from porcine intestinal mucosa: proposed derivation from the lactase-phlorizin hydrolase geneTropoelastin interacts with cell-surface glycosaminoglycans via its COOH-terminal domain.Elastin in lung development and disease pathogenesis.
P2860
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P2860
The 67-kDa enzymatically inactive alternatively spliced variant of beta-galactosidase is identical to the elastin/laminin-binding protein
description
1998 nî lūn-bûn
@nan
1998 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
1998 թվականի մարտին հրատարակված գիտական հոդված
@hy
1998年の論文
@ja
1998年学术文章
@wuu
1998年学术文章
@zh-cn
1998年学术文章
@zh-hans
1998年学术文章
@zh-my
1998年学术文章
@zh-sg
1998年學術文章
@yue
name
The 67-kDa enzymatically inact ...... lastin/laminin-binding protein
@ast
The 67-kDa enzymatically inact ...... lastin/laminin-binding protein
@en
The 67-kDa enzymatically inact ...... lastin/laminin-binding protein
@nl
type
label
The 67-kDa enzymatically inact ...... lastin/laminin-binding protein
@ast
The 67-kDa enzymatically inact ...... lastin/laminin-binding protein
@en
The 67-kDa enzymatically inact ...... lastin/laminin-binding protein
@nl
prefLabel
The 67-kDa enzymatically inact ...... lastin/laminin-binding protein
@ast
The 67-kDa enzymatically inact ...... lastin/laminin-binding protein
@en
The 67-kDa enzymatically inact ...... lastin/laminin-binding protein
@nl
P2093
P2860
P356
P1476
The 67-kDa enzymatically inact ...... lastin/laminin-binding protein
@en
P2093
J W Callahan
S Privitera
P2860
P304
P356
10.1074/JBC.273.11.6319
P407
P577
1998-03-13T00:00:00Z