Published in Kidney Int on January 08, 2014
Spotlights on immunological effects of reactive nitrogen species: When inflammation says nitric oxide. World J Exp Med (2015) 0.97
The balance of powers: Redox regulation of fibrogenic pathways in kidney injury. Redox Biol (2015) 0.91
Impact of gestational bisphenol A on oxidative stress and free fatty acids: Human association and interspecies animal testing studies. Endocrinology (2015) 0.82
3-Nitrotyrosine as a biomarker for vascular involvement in Fabry disease. Kidney Int (2014) 0.77
Innate and Adaptive Immune Response in Fabry Disease. JIMD Rep (2015) 0.75
Development of a model system for neuronal dysfunction in Fabry disease. Mol Genet Metab (2016) 0.75
Targeting Glycosphingolipid Metabolism to Treat Kidney Disease. Nephron (2016) 0.75
Serum Bilirubin Levels and Promoter Variations in HMOX1 and UGT1A1 Genes in Patients with Fabry Disease. Oxid Med Cell Longev (2017) 0.75
Functions of lysosomes. Annu Rev Physiol (1966) 17.76
Permanent cell line expressing human factor VIII-related antigen established by hybridization. Proc Natl Acad Sci U S A (1983) 7.55
Enzymatic defect in Fabry's disease. Ceramidetrihexosidase deficiency. N Engl J Med (1967) 5.63
Biological tyrosine nitration: a pathophysiological function of nitric oxide and reactive oxygen species. Arch Biochem Biophys (1998) 3.21
Elevated globotriaosylsphingosine is a hallmark of Fabry disease. Proc Natl Acad Sci U S A (2008) 2.87
Regulation of endothelial nitric oxide synthase: location, location, location. Annu Rev Physiol (2002) 2.79
Sustained, long-term renal stabilization after 54 months of agalsidase beta therapy in patients with Fabry disease. J Am Soc Nephrol (2007) 2.73
Fabry's disease: alpha-galactosidase deficiency. Science (1970) 2.67
Long-term safety and efficacy of enzyme replacement therapy for Fabry disease. Am J Hum Genet (2004) 2.60
FABRY'S DISEASE: CLASSIFICATION AS A SPHINGOLIPIDOSIS AND PARTIAL CHARACTERIZATION OF A NOVEL GLYCOLIPID. J Biol Chem (1963) 2.24
Association of nitrotyrosine levels with cardiovascular disease and modulation by statin therapy. JAMA (2003) 2.16
Differential assay for lysosomal alpha-galactosidases in human tissues and its application to Fabry's disease. Clin Chim Acta (1981) 2.01
Human atherosclerotic intima and blood of patients with established coronary artery disease contain high density lipoprotein damaged by reactive nitrogen species. J Biol Chem (2004) 1.68
Enhanced endothelium-dependent vasodilation in Fabry disease. Stroke (2001) 1.56
Tackling endothelial dysfunction by modulating NOS uncoupling: new insights into its pathogenesis and therapeutic possibilities. Am J Physiol Endocrinol Metab (2011) 1.37
Alpha-galactosidase A deficiency accelerates atherosclerosis in mice with apolipoprotein E deficiency. Circulation (2005) 1.28
Globotriaosylceramide induces oxidative stress and up-regulates cell adhesion molecule expression in Fabry disease endothelial cells. Mol Genet Metab (2008) 1.21
A systematic review on screening for Fabry disease: prevalence of individuals with genetic variants of unknown significance. J Med Genet (2013) 1.19
Endothelium specific Weibel-Palade bodies in a continuous human cell line, EA.hy926. In Vitro Cell Dev Biol (1990) 1.17
Fabry disease in mice is associated with age-dependent susceptibility to vascular thrombosis. J Am Soc Nephrol (2003) 1.15
Elevated cerebral blood flow velocities in Fabry disease with reversal after enzyme replacement. Stroke (2002) 1.10
Enzyme replacement therapy for Anderson-Fabry disease. Cochrane Database Syst Rev (2013) 1.09
Decreased nitric oxide bioavailability in a mouse model of Fabry disease. J Am Soc Nephrol (2009) 1.09
Vasculopathy in patients with Fabry disease: current controversies and research directions. Mol Genet Metab (2009) 1.08
Oxidized amino acids: culprits in human atherosclerosis and indicators of oxidative stress. Free Radic Biol Med (2002) 1.02
Caveolin-associated accumulation of globotriaosylceramide in the vascular endothelium of alpha-galactosidase A null mice. J Biol Chem (2007) 1.02
An in vitro model of Fabry disease. J Am Soc Nephrol (2005) 1.00
Quantitative analysis of amino Acid oxidation markers by tandem mass spectrometry. Methods Enzymol (2011) 0.99
Vascular dysfunction in the alpha-galactosidase A-knockout mouse is an endothelial cell-, plasma membrane-based defect. Clin Exp Pharmacol Physiol (2008) 0.99
Structural and functional changes in peripheral vasculature of Fabry patients. J Inherit Metab Dis (2006) 0.99
Quantification of globotriaosylsphingosine in plasma and urine of fabry patients by stable isotope ultraperformance liquid chromatography-tandem mass spectrometry. Clin Chem (2012) 0.98
ANGIOKERATOMA CORPORIS DIFFUSUM (FABRY). HISTOCHEMICAL AND ELECTRON MICROSCOPIC STUDIES OF THE SKIN. J Invest Dermatol (1965) 0.94
Reduced cerebral blood flow velocity and impaired cerebral autoregulation in patients with Fabry disease. J Neurol (2004) 0.90
Impaired myocardial perfusion reserve but preserved peripheral endothelial function in patients with Fabry disease. J Inherit Metab Dis (2005) 0.89
Peripheral hemodynamics in patients with Fabry's disease. Am Heart J (1983) 0.85
Vascular aspects of Fabry disease in relation to clinical manifestations and elevations in plasma globotriaosylsphingosine. Hypertension (2012) 0.83
Reduced coronary flow reserve in Anderson-Fabry disease measured by transthoracic Doppler echocardiography. Cardiovasc Ultrasound (2005) 0.83
Glycosphingolipid Mediated Caveolin-1 Oligomerization. J Glycomics Lipidomics (2012) 0.79
Non invasive evaluation of endothelial function in patients with Anderson-Fabry disease. Int Angiol (2005) 0.79
Postischemic cutaneous hyperperfusion in the presence of forearm hypoperfusion suggests sympathetic vasomotor dysfunction in Fabry disease. J Neurol (2003) 0.78
Biochemical response to substrate reduction therapy versus enzyme replacement therapy in Gaucher disease type 1 patients. Orphanet J Rare Dis (2016) 0.79