Cellular and tissue localization of globotriaosylceramide in Fabry disease. - Wikidata - wikimedia - TriplyDB

Cellular and tissue localization of globotriaosylceramide in Fabry disease.

Cellular and tissue localization of globotriaosylceramide in Fabry disease.

http://www.wikidata.org/entity/Q51979382

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Safety and efficacy of enzyme replacement therapy in the nephropathy of Fabry disease Understanding the gastrointestinal manifestations of Fabry disease: promoting prompt diagnosis Podocyte pathology and nephropathy - sphingolipids in glomerular diseases Oral Migalastat HCl Leads to Greater Systemic Exposure and Tissue Levels of Active α-Galactosidase A in Fabry Patients when Co-Administered with Infused Agalsidase Expression of uPAR in Urinary Podocytes of Patients with Fabry Disease.The pharmacological chaperone 1-deoxygalactonojirimycin reduces tissue globotriaosylceramide levels in a mouse model of Fabry disease.Small molecule structure correctors abolish detrimental effects of apolipoprotein E4 in cultured neurons.Globotriaosylsphingosine accumulation and not alpha-galactosidase-A deficiency causes endothelial dysfunction in Fabry disease.Alteration of proteomic profiles in PBMC isolated from patients with Fabry disease: preliminary findings.Migalastat HCl reduces globotriaosylsphingosine (lyso-Gb3) in Fabry transgenic mice and in the plasma of Fabry patients.Epithelial-Mesenchymal Transition in Kidney Tubular Epithelial Cells Induced by Globotriaosylsphingosine and Globotriaosylceramide Pharmacological chaperone therapy for Fabry disease Increased glycolipid storage produced by the inheritance of a complex intronic haplotype in the α-galactosidase A (GLA) gene.Proteomics and glomerulonephritis: A complementary approach in renal pathology for the identification of chronic kidney disease related markers.Skin Globotriaosylceramide 3 Load Is Increased in Men with Advanced Fabry Disease.Carpal tunnel syndrome in fabry disease.Elevated globotriaosylsphingosine is a hallmark of Fabry disease.Myofilament degradation and dysfunction of human cardiomyocytes in Fabry disease.Coformulation of a Novel Human α-Galactosidase A With the Pharmacological Chaperone AT1001 Leads to Improved Substrate Reduction in Fabry Mice Fibrosis: a key feature of Fabry disease with potential therapeutic implications Renal complications of Fabry disease in children Vascular tortuosities of the upper eyelid: a new clinical finding in fabry patient screening.A Short Synthetic Peptide Mimetic of Apolipoprotein A1 Mediates Cholesterol and Globotriaosylceramide Efflux from Fabry Fibroblasts.Autophagy-lysosome pathway associated neuropathology and axonal degeneration in the brains of alpha-galactosidase A-deficient mice.Altered dynamics of a lipid raft associated protein in a kidney model of Fabry disease Extracellular matrix components: an intricate network of possible biomarkers for lysosomal storage disorders?Influence of length and conformation of saccharide head groups on the mechanics of glycolipid membranes: Unraveled by off-specular neutron scattering.Is it Fabry disease?Increased urinary CD80 excretion and podocyturia in Fabry disease.Co-administration with the pharmacological chaperone AT1001 increases recombinant human α-galactosidase A tissue uptake and improves substrate reduction in Fabry mice.Sphingolipids and Redox Signaling in Renal Regulation and Chronic Kidney Diseases.Translational Aspects of Sphingolipid Metabolism in Renal Disorders.Long-term enzyme replacement therapy for Fabry disease: efficacy and unmet needs in cardiac and renal outcomes.Immunohistochemical diagnosis of Fabry nephropathy and localisation of globotriaosylceramide deposits in paraffin-embedded kidney tissue sections.Clinical significance of plasma globotriaosylsphingosine levels in Chinese patients with Fabry disease.Medullary thick ascending limb impairment in the GlatmTg(CAG-A4GALT) Fabry model mice.Globotriaosylsphingosine actions on human glomerular podocytes: implications for Fabry nephropathy.

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P2860

Cellular and tissue localization of globotriaosylceramide in Fabry disease.

http://www.wikidata.org/entity/Q51979382

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2007 nî lūn-bûn

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2007年學術文章

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name

Cellular and tissue localization of globotriaosylceramide in Fabry disease.

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Cellular and tissue localization of globotriaosylceramide in Fabry disease.

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Cellular and tissue localization of globotriaosylceramide in Fabry disease.

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Cellular and tissue localization of globotriaosylceramide in Fabry disease.

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Cellular and tissue localization of globotriaosylceramide in Fabry disease.

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Cellular and tissue localization of globotriaosylceramide in Fabry disease.

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Virchows Archiv

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Cellular and tissue localization of globotriaosylceramide in Fabry disease

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Agnes Ang

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Brandon A Wustman

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Cristina Semino-Mora

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Hasan Askari

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Linda E Spollen

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Lorah T Perlee

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Martha Quezado

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Priya Desai

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Raphael Schiffmann

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P2860

Enhanced endothelium-dependent vasodilation in Fabry disease

Effect of tissue processing on colloidal gold cytochemistry.

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s00428-007-0468-6

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823-834

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10.1007/S00428-007-0468-6

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David E. Kleiner

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2007-08-03T00:00:00Z

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α-D-galactosyl-(1→4)-β-D-galactosyl-(1→4)-β-D-glucosylceramide