Globotriaosylceramide induces oxidative stress and up-regulates cell adhesion molecule expression in Fabry disease endothelial cells.

No abstract is available for this paper.

• Publication year

  2008

• Venue

  Molecular Genetics and Metabolism

• Publication date

  2008-11-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1016/j.ymgme.2008.06.016PMID 18707907PMCID PMC2593623
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Elevated globotriaosylsphingosine is a hallmark of Fabry disease

  2008cited by this paper
• Elevated endothelial microparticles in Fabry children decreased after enzyme replacement therapy.

  2007cited by this paper
• Caveolin-associated Accumulation of Globotriaosylceramide in the Vascular Endothelium of α-Galactosidase A Null Mice*

  2007cited by this paper
• Establishment and characterization of Fabry disease endothelial cells with an extended lifespan.

  2007cited by this paper
• The cerebral vasculopathy of Fabry disease.

  2007cited by this paper
• Severe endothelial dysfunction in the aorta of a mouse model of Fabry disease; partial prevention by N-butyldeoxynojirimycin treatment

  2007cited by this paper
• Myeloperoxidase predicts risk of vasculopathic events in hemizgygous males with Fabry disease

  2006cited by this paper
• Enzyme-Replacement Therapy With Agalsidase Alfa in Children With Fabry Disease

  2006cited by this paper
• &agr;-Galactosidase A Deficiency Accelerates Atherosclerosis in Mice With Apolipoprotein E Deficiency

  2005cited by this paper
• Prevalence of Fabry disease in patients with cryptogenic stroke: a prospective study.

  2005cited by this paper
• Oxidative Stress and Vascular Disease

  2004cited by this paper
• CNS involvement in Fabry disease: Clinical and imaging studies before and after 12 months of enzyme replacement therapy

  2004cited by this paper
• Fabry disease: overall effects of agalsidase alfa treatment

  2004cited by this paper
• Long-term safety and efficacy of enzyme replacement therapy for Fabry disease.

  2004cited by this paper
• Ascorbate decreases Fabry cerebral hyperperfusion suggesting a reactive oxygen species abnormality: An arterial spin tagging study

  2004cited by this paper
• Role of oxidative modifications in atherosclerosis.

  2004cited by this paper
• Fabry disease in mice is associated with age-dependent susceptibility to vascular thrombosis.

  2003cited by this paper
• Adhesion molecules and atherosclerosis.

  2003cited by this paper
• The Metabolic and Molecular Bases of Inherited Disease

  2001cited by this paper
• Enhanced Endothelium-Dependent Vasodilation in Fabry Disease

  2001cited by this paper
• α-Galactosidase A deficiency. Fabry disease

  2001cited by this paper
• Regional Cerebral Hyperperfusion and Nitric Oxide Pathway Dysregulation in Fabry Disease: Reversal by Enzyme Replacement Therapy

  2001cited by this paper
• Multiple role of reactive oxygen species in the arterial wall

  2001cited by this paper
• Clinical features of and recent advances in therapy for Fabry disease.

  2000cited by this paper
• Superoxide Production and Reactive Oxygen Species Signaling by Endothelial Nitric-oxide Synthase*

  2000cited by this paper
• Profile of endothelial and leukocyte activation in fabry patients

  2000cited by this paper
• Improved Inhibitors of Glucosylceramide Synthase*

  1999cited by this paper
• Superoxide generation by endothelial nitric oxide synthase: the influence of cofactors.

  1998cited by this paper
• Superoxide Generation from Endothelial Nitric-oxide Synthase

  1998cited by this paper
• Cerebrovascular complications of Fabry's disease

  1996cited by this paper
• Fluorometric detection of glycosphingolipids on thin-layer chromatographic plates.

  1995cited by this paper
• Transcriptional regulation of endothelial cell adhesion molecules: NF‐κB and cytokine‐inducible enhancers

  1995cited by this paper
• Improved inhibitors of glucosylceramide synthase.

  1992cited by this paper
• E-selectin and intercellular adhesion molecule-1 are released by activated human endothelial cells in vitro.

  1992cited by this paper
• Uptake and metabolism of a fluorescent sulfatide analogue in cultured skin fibroblasts.

  1992cited by this paper
• Quantitative analysis of plasma neutral glycosphingolipids by high performance liquid chromatography of their perbenzoyl derivatives.

  1977cited by this paper
• Enzymatic defect in Fabry's disease. Ceramidetrihexosidase deficiency.

  1967cited by this paper

• Endothelial dysfunction in Fabry disease: retinal biomarkers link cardiac GLA gene variants with chronic inflammation

  2026cites this paper
• Impact of COVID-19 infection in patients with inherited metabolic diseases: a National Multicenter Study from the French IMDs Healthcare Network for Rare Diseases

  2026cites this paper
• Therapeutic effects of lomerizine on vasculopathy in Fabry disease

  2025cites this paper
• Telomere Length, Oxidative Stress, and Kidney Damage Biomarkers in Fabry Nephropathy

  2025cites this paper
• Imbalance in redox homeostasis is associated with neurodegeneration in the murine model of Tay-Sachs disease

  2025cites this paper
• Systemic inflammation in Fabry disease: a longitudinal immuno-genetic analysis based on variant stratification

  2025cites this paper
• Cardiac involvement in Fabry disease: Implications of retinal vessel analysis in detecting early microvascular alterations despite ERT

  2025cites this paper
• Fabry Disease Beyond Storage: The Role of Inflammation in Disease Progression

  2025cites this paper
• A4GALT-targeting siRNA lipid nanoparticles ameliorate Fabry disease phenotype: Greater efficacy in endothelial cells than in podocytes

  2025cites this paper
• Enhancement of Maturation and Functionality of Human Fabry Disease Kidney Organoids in a Microfluidic Gradient Chip

  2025cites this paper
• Renoprotective effects of SGLT2 inhibitors in patients with Fabry disease

  2025cites this paper
• Cold-evoked potentials in Fabry disease and polyneuropathy

  2024cites this paper
• Long-term enzyme replacement therapy in Fabry patients protects against oxidative and inflammatory process

  2024cites this paper
• Inflammation in Fabry disease: stages, molecular pathways, and therapeutic implications

  2024cites this paper
• Neuropathy and pain in Fabry disease

  2024cites this paper
• Systematic metabolomics study in the serum and urine of a mouse model of Fabry disease

  2024cites this paper
• Genome-wide expression analysis in a Fabry disease human podocyte cell line

  2024cites this paper
• Cerebrovascular disorders and Fabry disease

  2024cites this paper
• Targeted nanoliposomes to improve enzyme replacement therapy of Fabry disease

  2024cites this paper
• Inflammation, Oxidative Stress, and Endothelial Dysfunction in the Pathogenesis of Vascular Damage: Unraveling Novel Cardiovascular Risk Factors in Fabry Disease

  2024influential citation
• Overexpression of VEGFα as a biomarker of endothelial dysfunction in aortic tissue of α-GAL-Tg/KO mice and its upregulation in the serum of patients with Fabry’s disease

  2024cites this paper
• Ventricular arrhythmias and primary prevention of sudden cardiac death in Anderson-Fabry disease.

  2024cites this paper
• Relationship between Capillaroscopic Architectural Patterns and Different Variant Subgroups in Fabry Disease: Analysis of Cases from a Multidisciplinary Center

  2024cites this paper
• Complement System and Adhesion Molecule Skirmishes in Fabry Disease: Insights into Pathogenesis and Disease Mechanisms

  2024cites this paper
• Cardiopulmonary determinants of reduced exercise tolerance in Fabry disease

  2024cites this paper
• Investigating Fabry disease - some lessons learned

  2024cites this paper
• Characterization of the Plasma Proteomic Profile of Fabry Disease: Potential Sex- and Clinical Phenotype-specific Biomarkers.

  2024cites this paper
• Prevalence of Fabry disease and GLA variants in young patients with acute stroke: The challenge to widen the screening. The Fabry-Stroke Italian Registry.

  2024cites this paper
• Oxidative stress and its role in Fabry disease

  2024cites this paper
• Inflammatory and Cardiovascular Biomarkers to Monitor Fabry Disease Progression

  2024cites this paper
• Inflammation and Exosomes in Fabry Disease Pathogenesis

  2024influential citation
• Screening and diagnosis of Fabry disease in chronic kidney disease: the important role of globotriaosylsphingosine

  2024cites this paper
• Screening for Fabry Disease-Related Mutations Among 829 Kidney Transplant Recipients

  2024cites this paper
• Exosome Secretion and Cellular Signaling Change in a Fabry Disease Cell Model Induced by Gene-silencing

  2024cites this paper
• FABRY DISEASE MANAGEMENT: CURRENT STATUS, THERAPEUTIC CHALLENGES, AND FUTURE HORIZONS IN DRUG DELIVERY AND ARTIFICIAL INTELLIGENCE ASSISTED DIAGNOSIS

  2024cites this paper
• Gene therapy with lipid nanoparticles for Fabry disease: Leveraging E-selectin for targeted delivery to endothelial cells

  2024cites this paper
• Evaluating the Metabolic Basis of α-Gal A mRNA Therapy for Fabry Disease

  2024cites this paper
• Management of Hypertension in Fabry Disease

  2023cites this paper
• Inflammation across the spectrum of hypertrophic cardiac phenotypes

  2023cites this paper
• Biochemical Mechanisms beyond Glycosphingolipid Accumulation in Fabry Disease: Might They Provide Additional Therapeutic Treatments?

  2023cites this paper
• Endocrinological, immunological and metabolic features of patients with Fabry disease under therapy

  2023cites this paper
• From bedside to the bench: patient-specific hiPSC-EC models uncover endothelial dysfunction in genetic cardiomyopathies

  2023influential citation
• Challenging the Conventional Treatment Initiation Paradigm: Early Detection of Irreversible Cellular Damage in Cardiac Biopsies of Fabry Disease Before the Formation of Gb3 Inclusion Bodies

  2023cites this paper
• Urinary-derived extracellular vesicles reveal a distinct microRNA signature associated with the development and progression of Fabry nephropathy

  2023cites this paper
• Vitamin C Urinary Loss in Fabry Disease: Clinical and Genomic Characteristics of Vitamin C Renal Leak.

  2023cites this paper
• Accumulation of α-synuclein mediates podocyte injury in Fabry nephropathy

  2023cites this paper
• Proteomic analysis unveils Gb3-independent alterations and mitochondrial dysfunction in a gla−/− zebrafish model of Fabry disease

  2023cites this paper
• Molecular Pathogenesis of Central and Peripheral Nervous System Complications in Anderson–Fabry Disease

  2023cites this paper
• Lipidomic Approaches in Common and Rare Cerebrovascular Diseases: The Discovery of Unconventional Lipids as Novel Biomarkers

  2023influential citation
• Fabry Disease in Women: Genetic Basis, Available Biomarkers, and Clinical Manifestations

  2023cites this paper
• Exploring Pro-Inflammatory Immunological Mediators: Unraveling the Mechanisms of Neuroinflammation in Lysosomal Storage Diseases

  2023cites this paper
• Thiol/disulfide homeostasis as a new oxidative stress marker in patients with Fabry disease

  2023cites this paper
• Investigation of OCTA Biomarkers in Fabry Disease: A Long Term Follow-Up of Macular Vessel Area Density and Foveal Avascular Zone Metrics

  2023cites this paper
• Effects of Natural Polyphenols on Oxidative Stress-Mediated Blood-Brain Barrier Dysfunction

  2022cites this paper
• Growth Differentiation Factor-15 and Syndecan-1 Are Potential Biomarkers of Cardiac and Renal Involvement in Classical Fabry Disease under Enzyme Replacement Therapy

  2022cites this paper
• Gene Expression Analysis in gla-Mutant Zebrafish Reveals Enhanced Ca2+ Signaling Similar to Fabry Disease

  2022cites this paper
• Prediction of Regulatory SNPs in Putative Minor Genes of the Neuro-Cardiovascular Variant in Fabry Reveals Insights into Autophagy/Apoptosis and Fibrosis

  2022cites this paper
• Characterization of cellular phenotypes in neurons derived from induced pluripotent stem cells of male patients with Fabry disease

  2022cites this paper
• Clinical relevance of globotriaosylceramide accumulation in Fabry disease and the effect of agalsidase beta in affected tissues.

  2022cites this paper
• Inhibition of epigenetic reader proteins by apabetalone counters inflammation in activated innate immune cells from Fabry disease patients receiving enzyme replacement therapy

  2022cites this paper
• Ceria-Zirconia nanoparticles reduce intracellular globotriaosylceramide accumulation and attenuate kidney injury by enhancing the autophagy flux in cellular and animal models of Fabry disease

  2022cites this paper
• X-Chromosome Inactivation and Related Diseases

  2022cites this paper
• Review of Mechanisms, Pharmacological Management, Psychosocial Implications, and Holistic Treatment of Pain in Fabry Disease

  2021influential citation
• Defective lysosomal storage in Fabry disease modifies mitochondrial structure, metabolism and turnover in renal epithelial cells

  2021influential citation
• Reversal of the Inflammatory Responses in Fabry Patient iPSC-Derived Cardiovascular Endothelial Cells by CRISPR/Cas9-Corrected Mutation

  2021cites this paper
• Fabry Disease: Molecular Basis, Pathophysiology, Diagnostics and Potential Therapeutic Directions

  2021cites this paper
• Synuclein alpha accumulation mediates podocyte injury in Fabry nephropathy

  2021cites this paper
• Pathology and pathogenic pathways in fabry nephropathy

  2021cites this paper
• Biomarkers in Fabry Disease. Implications for Clinical Diagnosis and Follow-up

  2021cites this paper
• Arterial involvement in Fabry disease: state of the art and future diagnostic purposes.

  2021cites this paper
• Parsing Fabry Disease Metabolic Plasticity Using Metabolomics

  2021cites this paper
• Fabry Cardiomyopathy: Current Treatment and Future Options

  2021influential citation
• X Chromosome Inactivation in Carriers of Fabry Disease: Review and Meta-Analysis

  2021cites this paper
• Gene therapy for Fabry disease: Progress, challenges, and outlooks on gene-editing.

  2021cites this paper
• Anderson–Fabry Disease: From Endothelial Dysfunction to Emerging Therapies

  2021cites this paper
• MiRNA Let-7a and Let-7d Are Induced by Globotriaosylceramide via NF-kB Activation in Fabry Disease

  2021cites this paper
• RIPK3 Contributes to Lyso-Gb3-Induced Podocyte Death

  2021cites this paper
• Enfermedad de Fabry y embarazo. Situación actual y revisión de la evidencia

  2021cites this paper
• Accumulation of Seminolipid in Sertoli Cells Is Associated with Increased Levels of Reactive Oxygen Species and Male Subfertility: Studies in Aging Arsa Null Male Mice

  2021cites this paper
• Increased Serum Interleukin-6 and Tumor Necrosis Factor Alpha Levels in Fabry Disease: Correlation with Disease Burden

  2021cites this paper
• Chloroquine may induce endothelial injury through lysosomal dysfunction and oxidative stress

  2021cites this paper
• The Cardiovascular Phenotype in Fabry Disease: New Findings in the Research Field

  2021cites this paper
• Can Be miR-126-3p a Biomarker of Premature Aging? An Ex Vivo and In Vitro Study in Fabry Disease

  2021cites this paper
• Fabry Disease and the Heart: A Comprehensive Review

  2021cites this paper
• Fabry disease exacerbates renal interstitial fibrosis after unilateral ureteral obstruction via impaired autophagy and enhanced apoptosis

  2021cites this paper
• Nanotechnology-based approaches for treating lysosomal storage disorders, a focus on Fabry disease.

  2020cites this paper
• Does left ventricular function predict cardiac outcome in Anderson–Fabry disease?

  2020cites this paper
• Fabry disease patients have an increased risk of stroke in the COVID-19 ERA. A hypothesis

  2020cites this paper
• The role of Immunity in Fabry Disease and Hypertension: A Review of a Novel Common Pathway

  2020cites this paper
• Characteristics of Vascular Phenotype in Fabry Patients

  2020influential citation
• Oxidative stress biomarkers in Fabry disease: is there a room for them?

  2020cites this paper
• Left ventricular radial strain impairment precedes hypertrophy in Anderson–Fabry disease

  2020cites this paper
• Mechanisms of Mitochondrial Dysfunction in Lysosomal Storage Disorders: A Review

  2020cites this paper
• Progress in the understanding and treatment of Fabry disease.

  2020cites this paper
• Plasma lipidomic analysis of sphingolipids in patients with large artery atherosclerosis cerebrovascular disease and cerebral small vessel disease

  2020cites this paper
• Ion channels as potential redox sensors in lysosomes

  2019cites this paper
• Unexpected, But Reasonable Association Between Anderson-Fabry Disease and Coronary Vasospasm.

  2019cites this paper
• α-galactosidase A deficiency promotes von Willebrand factor secretion in models of Fabry disease.

  2019cites this paper
• Role of Resveratrol and Selenium on Oxidative Stress and Expression of Antioxidant and Anti-Aging Genes in Immortalized Lymphocytes from Alzheimer’s Disease Patients

  2019cites this paper
• Hsp 70 interactions with membrane lipids regulate cellular functions in health and disease

  2019cites this paper