Keywords: گلوتروئیوسیلسرامید; Fabry disease; Diagnosis; Mutation; Management; Treatment; ACEI; angiotensin converting enzyme inhibitor; ARB; angiotensin receptor blocker; AV; atrioventricular; α-Gal A; α-galactosidase A; CKD; chronic kidney disease; CNS; central nervous system; CT;
مقالات ISI گلوتروئیوسیلسرامید (ترجمه نشده)
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Keywords: گلوتروئیوسیلسرامید; α-GalA; α-galactosidase A; eGFR; estimated glomerular filtration rate; E/Ea; estimated left ventricular filling pressure; FD; Fabry disease; Gb3; globotriaosylceramide; IVS; interventricular septal; LV; left ventricular; LC-ESI-MS/MS; liquid chromatogra
Keywords: گلوتروئیوسیلسرامید; Fabry disease; Meningioma; Globotriaosylceramide; Alpha-galactosidase A; Lysosomal storage disease; Cancer;
Keywords: گلوتروئیوسیلسرامید; ERT; enzyme replacement therapy; Gb3; Globotriaosylceramide; IVSd; diastolic interventricular septal thickness; LOD; limit of detection; LVIDd; diastolic left ventricular internal diameter; LVIDs; systolic left ventricular internal diameter; LVM; left ven
Keywords: گلوتروئیوسیلسرامید; BMP; bismonoacyl glycerophosphate; CE; cholesteryl ester; CHOL; cholesterol; Cer; ceramide; DAG; diacylglycerol; DRM; detergent resistant membrane; FCS; fetal calf serum; Gb3; globotriaosylceramide; GLC; gas liquid chromatography; HexCer; hexosylceramide;
Keywords: گلوتروئیوسیلسرامید; Lysosomal Storage Disorders; Oxidative stress; Reactive species; Oxidative damage; 4-HNE; 4-hydroxynonenal; 7-KC; 7-ketocholesterol; 8-OHdG; 8-hydroxy-2â²-deoxyguanosine; AGE; advanced glycation end product; BD; Batten disease; Ca2Â +; Â calcium; CAT; ca
Keywords: گلوتروئیوسیلسرامید; α-GlcChol; 1-O-cholesteryl-α-d-glucopyranoside; α-Syn; α-synuclein; β-GlcChol; 1-Î-cholesteryl-β-d-glucopyranoside or β-cholesterylglucoside; BMP; bis (monoacylglycero) phosphate; CERT; ceramide transfer protein; CNS; central nervous system; ER; e
Keywords: گلوتروئیوسیلسرامید; α-gal A; α-galactosidase A; Cr; creatinine; ERT; enzyme replacement therapy; FD; Fabry disease; GCL; glutamate cysteine ligase; Gb3; globotriaosylceramide; GLA; α-galactosidase A coding gene; GPx; glutathione peroxidase; GR; glutathione reductase; GSH;
Keywords: گلوتروئیوسیلسرامید; cardiac fibrosis; IVS4+919G>A; late gadolinium enhancement; newborn screening; CAD; coronary artery disease; ERT; enzyme replacement therapy; FD; Fabry disease; Gal A; galactosidase A; Gb3; globotriaosylceramide; GE-CMR; gadolinium-enhanced cardiac magnet
Keywords: گلوتروئیوسیلسرامید; MS; mass spectrometry; GSL; glycosphingolipid; Cer; ceramide; Gb3; globotriaosylceramide; GlcCer; glucosylceramide; LacCer; lactosylceramide; MRM; multiple reaction monitoring; lipid turnover; sphingolipids; mass spectrometry; mass isotopes; FLUX lipidomi
Keywords: گلوتروئیوسیلسرامید; Gb3; globotriaosylceramide; lyso-Gb3; globotriaosylsphingosine; α-GAL; alpha-galactosidase A; LVH; left ventricular hypertrophy; ERT; enzyme replacement therapy; CFDI; Canadian Fabry Disease Initiative; REB; Research Ethics Board; CHUS; Centre hospitalie
Keywords: گلوتروئیوسیلسرامید; endothelial cell; endothelial nitric oxide synthase; Fabry disease; globotriaosylceramide;
Keywords: گلوتروئیوسیلسرامید; Cer; ceramide; CE; cholesterol esters; DAG; diacylglycerol; ELISA; enzyme-linked immunesorbant assay; ER; endoplasmic reticulum; Gb3; globotriaosylceramide; GlcCer; glucosylceramide; HG; hexadecylglycerol; LacCer; lactosylceramide; MS; mass spectrometry;
Keywords: گلوتروئیوسیلسرامید; Fabry disease; Lysosomal storage disorders; Pharmacological chaperone; α-Galactosidase A; Globotriaosylceramide;
Keywords: گلوتروئیوسیلسرامید; LD; lysosomal disorder; PBMC; peripheral blood mononuclear cells; DC; dendritic cells; MΦ; macrophage; NK; natural killer; Gb3; globotriaosylceramide; lysoGb3; globotriaosylsphingosine; αGal; α-galactosidase A; GLA (Gla); human (murine) α-galactosidas
Generation of Fabry cardiomyopathy model for drug screening using induced pluripotent stem cell-derived cardiomyocytes from a female Fabry patient
Keywords: گلوتروئیوسیلسرامید; RFLP; restriction fragment length polymorphism; TUNEL; TdT-mediated dUTP nick end labeling; PBMC; peripheral blood mononuclear cell; MEF; mouse embryonic fibroblast; BSA; bovine serum albumin; Fabry disease; Fabry cardiomyopathy; Induced pluripotent stem
Human monocytes stimulated by Shiga toxin 1a via globotriaosylceramide release proinflammatory molecules associated with hemolytic uremic syndrome
Keywords: گلوتروئیوسیلسرامید; Gb3Cer; globotriaosylceramide; HC; hemorrhagic colitis; HUS; hemolytic uremic syndrome; Stx; Shiga toxins; Stx1a; Shiga toxin 1a; Stx2a; Shiga toxin 2a; STEC; Shiga toxins-producing Escherichia coli; TLR4; toll-like receptor 4; Proinflammatory mediators;
The lysosomal enzyme alpha-Galactosidase A is deficient in Parkinson's disease brain in association with the pathologic accumulation of alpha-synuclein
Keywords: گلوتروئیوسیلسرامید; 4-MUG; 4-methylumbelliferone α-d-galactopyranoside; GalNAc; N-acetyl-d-galactosamine; α-Gal A; alpha-Galactosidase A; α-syn; alpha-synuclein; ALP; autophagy-lysosome pathway; Cat B; cathepsin B; Cat D; cathepsin D; Gb3; globotriaosylceramide; lyso-Gb3;
The severe clinical phenotype for a heterozygous Fabry female patient correlates to the methylation of non-mutated allele associated with chromosome 10q26 deletion syndrome
Keywords: گلوتروئیوسیلسرامید; α-gal A; α-galactosidase A; Gb3; globotriaosylceramide; Gb2; galabiosylceramide; XCI; X-chromosomal inactivation; LVH; left ventricular hypertrophy; α-Galactosidase A; Globotriaosylceramide; Intellectual disability; X-chromosomal inactivation; Methylat
Sialidase NEU3 defines invasive potential of human glioblastoma cells by regulating calpain-mediated proteolysis of focal adhesion proteins
Keywords: گلوتروئیوسیلسرامید; FAK; Focal adhesion kinase; FA; Focal adhesion; ECM; Extracellular matrix; Suc-LLVY-AMC; Succinyl-L-leucyl-L-leucyl-L-valyl-L-tyrosine 4-methylcoumaryl-7-amide; PIP2; Phosphatidylinositol 4, 5-bisphosphate; TLC; Thin-layer chromatography; Sulfo-NHS-biotin
Immunization with BLS-Stx2B chimera totally protects dams from early pregnancy loss induced by Shiga toxin type 2 (Stx2) and confers anti-Stx2 immunity to the offspring
Keywords: گلوتروئیوسیلسرامید; ANOVA; analysis of variance; BLS; lumazine synthase from Brucella spp.; CD50; 50% cytotoxic dose; DMEM; Dulbecco's Modified Eagle's Medium; FBS; fetal bovine serum; gd; gestation day; Gb3; globotriaosylceramide; HUS; Hemolytic Uremic Syndrome; i.p.; i
Tandem mass spectrometry multiplex analysis of methylated and non-methylated urinary Gb3 isoforms in Fabry disease patients
Keywords: گلوتروئیوسیلسرامید; OMIM; Online Mendelian Inheritance in Man; α-GAL A; α-galactosidase A; ERT; enzyme replacement therapy; CFDI; Canadian Fabry Disease Initiative; Gb3 or GL-3; globotriaosylceramide; CTH; ceramide trihexoside; Ga2; galabiosylceramide; CDH; ceramide dihexo
Risk factors for severe clinical events in male and female patients with Fabry disease treated with agalsidase beta enzyme replacement therapy: Data from the Fabry Registry
Keywords: گلوتروئیوسیلسرامید; eGFR; estimated glomerular filtration rate; ERT; enzyme replacement therapy; ESRD; end-stage renal disease; GL-3; globotriaosylceramide; IEC; independent ethics committee; IRB; institutional review board; LVH; left ventricular hypertrophy; UP/Cr; urinary
Accurate quantification of sphingosine-1-phosphate in normal and Fabry disease plasma, cells and tissues by LC-MS/MS with 13C-encoded natural S1P as internal standard
Keywords: گلوتروئیوسیلسرامید; 13C5-S1P; 13C5 C18-S1P; C18-S1P-ene; C18-S1P-diene; Gb3; globotriaosylceramide; lyso-Gb3; globotriaosylsphingosine; DIPEA; N,N-diisopropylethylamine; SA-1P; sphinganine-1-phosphate; S1P; sphingosine-1-phosphate; SK; sphingosine kinases; Sphingolipid; Sphi
Genetic Screening of Anderson-Fabry Disease in Probands Referred From Multispecialty Clinics
Keywords: گلوتروئیوسیلسرامید; α-Gal; biochemical; family screening; GLA; MOGE(S) classification; multidisciplinary evaluation; AFD; Anderson-Fabry disease; Gal; galactosidase A (enzyme); Gb3; globotriaosylceramide; HCM; hypertrophic cardiomyopathy; LV; left ventricle; LVH; left ventr
The Fabry disease-associated lipid Lyso-Gb3 enhances voltage-gated calcium currents in sensory neurons and causes pain
Keywords: گلوتروئیوسیلسرامید; Gb3; globotriaosylceramide; Lyso-Gb3; lyso-globotriaosylceramide; GLA; gene encoding alpha galactosidase A; HBSS; HEPES buffered saline; PBS; phosphate buffered saline; Pain; Calcium imaging; Voltage-dependent Ca2+ channels; Fabry disease; Dorsal root gan
l-rhamnose-binding lectin from eggs of the Echinometra lucunter: Amino acid sequence and molecular modeling
Keywords: گلوتروئیوسیلسرامید; Lectin; Mass spectrometry; Marine invertebrate; Rhamnose; Globotriaosylceramide;
Targeting glucosylceramide synthase induction of cell surface globotriaosylceramide (Gb3) in acquired cisplatin-resistance of lung cancer and malignant pleural mesothelioma cells
Keywords: گلوتروئیوسیلسرامید; Gb3; Globotriaosylceramide; GCS; Glucosylceramide synthase; GSL; Glycosphingolipid; MDR1; Multidrug resistance 1/P-glycoprotein; MPM; Malignant pleural mesothelioma (MPM); MRP1; Multidrug resistance-associated protein 1; NSCLC; Non-small cell lung cancer;
Plasma globotriaosylsphingosine (lysoGb3) could be a biomarker for Fabry disease with a Chinese hotspot late-onset mutation (IVS4Â +Â 919G>A)
Keywords: گلوتروئیوسیلسرامید; Gb3; globotriaosylceramide; LysoGb3; globotriaosylsphingosine; LVMI; left ventricular mass index; LC-MS/MS; high performance liquid chromatography mass spectrometry; ERT; enzyme replacement therapy; Fabry disease; IVS4Â +Â 919G>A; LysoGb3; Newborn screeni
Mutant α-galactosidase A with M296I does not cause elevation of the plasma globotriaosylsphingosine level
Keywords: گلوتروئیوسیلسرامید; Fabry disease; α-Galactosidase A; Missense mutation; Globotriaosylceramide; Globotriaosylsphingosine; Structural modeling;
Histologic abnormalities of placental tissues in Fabry disease: a case report and review of the literature
Keywords: گلوتروئیوسیلسرامید; Fabry disease; Placenta; Globotriaosylceramide; α-Galactosidase A; Lysosomal storage disease
Novel alpha-galactosidase A mutation in a female with recurrent strokes
Keywords: گلوتروئیوسیلسرامید; AFD; Anderson-Fabry disease; GL-3; globotriaosylceramide; cDNA; complementary DNA; 3â² UTR; three prime untranslated region; pAS; polyadenylation signal; CT; computed tomography; magnetic resonance imaging; MRI; DBFP; Dried Blood Filter Paper; K,EDTA;
Fabry disease: Biochemical, pathological and structural studies of the α-galactosidase A with E66Q amino acid substitution
Keywords: گلوتروئیوسیلسرامید; Fabry disease; α-Galactosidase A; Globotriaosylceramide; Globotriaosylsphingosine; Structural modeling
Biomarkers for the mucopolysaccharidoses: Discovery and clinical utility
Keywords: گلوتروئیوسیلسرامید; 6MWT; six-minute walk test; BMP; bis(monoacylglycero)phosphate; CLN; ceroid lipofuscinosis; CSF; cerebral spinal fluid; DPP-IV; dipeptidyl peptidase IV; ESI-MS/MS; electrospray ionization-tandem mass spectrometry; ERT; enzyme replacement therapy; GAG; gly
Is complement a culprit in infection-induced forms of haemolytic uraemic syndrome?
Keywords: گلوتروئیوسیلسرامید; HUS; haemolytic uraemic syndrome; TMA; thrombotic microangiopathy; ECs; endothelial cells; AP; alternative pathway; CP; classical pathway; MAC; membrane attack complex; CFH; complement factor H; SCRs; short consensus repeats; CFI; complement factor I; DAF
Shiga toxins
Keywords: گلوتروئیوسیلسرامید; Shiga toxin; Verotoxin; Ribosome-inactivating proteins; Globotriaosylceramide;
Diagnostic dilemma: A young woman with Fabry disease symptoms, no family history, and a “sequencing cryptic” α-galactosidase a large deletion
Keywords: گلوتروئیوسیلسرامید; α-Gal A; α-galactosidase A; GL-3 or Gb3; globotriaosylceramide; ERT; enzyme replacement therapy; MLPA; Multiplex Ligation-dependent Probe Amplification; PCR; polymerase chain reaction; Fabry disease; Lysosomal storage disease; α-galactosidase A; Gene r
Higher apoptotic state in Fabry disease peripheral blood mononuclear cells.
Keywords: گلوتروئیوسیلسرامید; Gb3; globotriaosylceramide; LSD; lysosomal storage disorder; ER; endoplasmic reticulum; MPS; mucopolysaccharidosis; PBMC; peripheral blood mononuclear cells; MFI; mean fluorescence intensity; ERT; enzyme replacement therapy; GLA; α-galactosidase A; DGJ;
Sphingolipids and expression regulation of genes in cancer
Keywords: گلوتروئیوسیلسرامید; C1P; ceramide 1-phosphate; CAPP; ceramide-activated protein phosphatase; CerK; ceramide kinase; CerS; ceramide synthase; CERT; ceramide transfer protein; COX-2; cyclooxygenase-2; CPPase; ceramide phosphate phosphatase; CSC; cancer stem cell; ES; embryo st
Comparison of detection methods for cell surface globotriaosylceramide
Keywords: گلوتروئیوسیلسرامید; Alexa488; fluorescent dye derived from 3,6-diaminoxanthenium-4,5-disulfate; EBV; Epstein-Barr virus; FITC; fluorescein isothiocyanate; FSC; forward scatter; Gb3; globotriaosylceramide; GSL; glycosphingolipid; HIV; human immunodeficiency virus; HUS; hemo
Plasma globotriaosylsphingosine as a biomarker of Fabry disease
Keywords: گلوتروئیوسیلسرامید; Fabry disease; Globotriaosylsphingosine; Globotriaosylceramide; α-Galactosidase A; Biomarker; Enzyme replacement therapy
Tissue and plasma globotriaosylsphingosine could be a biomarker for assessing enzyme replacement therapy for Fabry disease
Keywords: گلوتروئیوسیلسرامید; Fabry disease; Globotriaosylsphingosine; Globotriaosylceramide; α-Galactosidase A; Enzyme replacement therapy; Biomarker
Inner ear pathology of alpha-galactosidase A deficient mice, a model of Fabry disease
Keywords: گلوتروئیوسیلسرامید; Alpha-galactosidase A deficient mouse; Globotriaosylceramide; Hearing loss; Cochlea histopathology;
Four-Year Prospective Clinical Trial of Agalsidase Alfa in Children with Fabry Disease
Keywords: گلوتروئیوسیلسرامید; BPI; Brief Pain Index; eGFR; Estimated glomerular filtration rate; Gb3; Globotriaosylceramide; LVM; Left ventricular mass;
An easy and sensitive method for determination of globotriaosylceramide (Gb3) from urinary sediment: Utility for Fabry disease diagnosis and treatment monitoring
Keywords: گلوتروئیوسیلسرامید; Gb3, globotriaosylceramide; α-Gal A, α-galactosidase A; ERT, enzyme replacement therapyFabry disease; Globotriaosylceramide; Urinary sediment; Diagnosis; Enzyme replacement therapy; Lysosomal storage disorders
Fabry disease urinary globotriaosylceramide/creatinine biomarker evaluation by liquid chromatography–tandem mass spectrometry in healthy infants from birth to 6 months
Keywords: گلوتروئیوسیلسرامید; Fabry disease; Globotriaosylceramide; Gb3; GL-3; CTH; Gb3/creatinine; Liquid chromatography–tandem mass spectrometry; LC–MS/MS; Urine filter paper sample; Screening; Biomarker
Binding of Silurus asotus lectin to Gb3 on Raji cells causes disappearance of membrane-bound form of HSP70
Keywords: گلوتروئیوسیلسرامید; Heat shock protein; Membrane-bound form; Cholesterol rich microdomain; Burkitt's lymphoma cell; Rhamnose-binding lectin; Catfish (Silurus asotus); Globotriaosylceramide;
A retrospective analysis of the potential impact of IgG antibodies to agalsidase β on efficacy during enzyme replacement therapy for Fabry disease
Keywords: گلوتروئیوسیلسرامید; Fabry disease; Agalsidase β; Lysosomal storage disorder; α-Galactosidase A; IgG antibodies; Enzyme replacement therapy; Globotriaosylceramide; X-linked disease
Guidelines to Diagnosis and Monitoring of Fabry Disease and Review of Treatment Experiences
Keywords: گلوتروئیوسیلسرامید; α-Gal A; α-Galactosidase A; CKD; Chronic kidney disease; eGFR; Estimated glomerular filtration rate; ERT; Enzyme replacement therapy; GLA; α-Galactosidase A gene; GL-3; Globotriaosylceramide; GL-2; Lactosylceramide; HDL; High-density lipoprotein; LDL;
Globotriaosylceramide induces oxidative stress and up-regulates cell adhesion molecule expression in Fabry disease endothelial cells
Keywords: گلوتروئیوسیلسرامید; Fabry disease; α-Galactosidase A deficiency; Globotriaosylceramide; Endothelial cells; Reactive oxygen species; Cell adhesion molecule