Keywords: هستند; 6-OHDA; 6-hydroxydopamine; ACE; angiotensin converting enzyme; ACEIs; ACE inhibitors; AII; angiotensin II; AT1; AII type 1 receptor; AT2; AII type 2 receptor; CNS; central nervous system; DA; dopamine; DPN; 2,3-Bis-4-hydroxyphenyl-propionitrile, ER-β ago
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Keywords: هستند; Turbulence; Quantification techniques; ERT; Piezoelectric sensors; Conductivity probe;
Keywords: هستند; BMI; Body mass index; DHEA; Dehydroepiandrosterone; ERT; Enzyme replacement therapy; GAA; Acid α-glucosidase; IPD; Infantile PD; PCOS; Polycystic ovary syndrome; PD; Pompe disease;
Keywords: هستند; C6S; chondroitin-6-sulfate; ERT; enzyme replacement therapy; GAGs; glycosaminoglycans; GALNS; the gene encoding N-acetylgalactosamine-6-sulfatase; GALNS; the protein N-acetylgalactosamine-6-sulfatase, also known as galactosamine-6-sulfate sulfatase; KS; k
Keywords: هستند; ERT; enzyme replacement therapy; CLN1; ceroid lipofuscinosis, neuronal-1; CLN2; ceroid lipofuscinosis, neuronal-2; LSD; lysosomal storage disorder; MU-6S-Palm-βGlc; 4-methylumbelliferyl-6-thiopalmitoyl-β-d-glucoside; NCL; neuronal ceroid lipofuscinosis;
Keywords: هستند; CK; Creatinine kinase; CRIM; Cross-reactive immunologic material; ERT; Enzyme-replacement therapy; GAA; Acid α-glucosidase; IOPD; Infantile-onset Pompe disease; LVMI; Left ventricular mass index; MRI; Magnetic resonance imaging; NBS; Newborn screening; P
Keywords: هستند; 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: هستند; Immunology; primary immunodeficiency; dedicator of cytokinesis 8; HIV1; recombination-activating gene 1; intravenous immunoglobulin; common variable immunodeficiency; severe combined immunodeficiency; newborn screening; ADA; Adenosine deaminase; ApoE; Apo
Keywords: هستند; Water injection test; ERT; Moment analysis; Unsaturated sandstone; Hydraulic conductivity
Keywords: هستند; GPR; Ground Penetrating Radar; ERT; Electrical Resistivity Tomography; AOD; above ordnance datum; Pingo; Lithalsa; Periglacial; Devensian; Geophysical;
Keywords: هستند; Jurassic Mudstone; Landslide; LiDAR; ERT;
Keywords: هستند; TEM; ERT; Seismic reflection; Seismic refraction; Hydrogeophysics; Okavango Delta;
Keywords: هستند; AAV; adeno-associated virus; CNS; central nervous system; DLB; dementia with Lewy bodies; ERAD; endoplasmic reticulum associated-degradation; ERT; enzyme replacement therapy; FDA; Food and Drug Administration; GBA1; lysosomal glucocerebrosidase gene; GBA2
Keywords: هستند; BL; baseline; BMB; bone marrow burden; BMD; bone mineral density; CCL18; chemokine (C-C motif) ligand 18; EOW; every other week; ERT; enzyme replacement therapy; F; femur; F-BMB; femoral bone marrow burden; FN; femoral neck; GD; Gaucher disease; LS; lum
Keywords: هستند; ERT; CO2 sequestration; Probabilistic inversion; Model parameterization;
Keywords: هستند; AE; Anomaly Effect; DZ; Dahlin and Zhou; ERT; Electrical Resistivity Tomography; PD; Pole-Dipole; PsPD; Pseudo-Pole-Dipole (the exact location of the remote electrode is used even finite); PDbias; Pole-Dipole bias (remote electrode is considered at
Keywords: هستند; Quarter 27; ERT; Seismic refraction; MASW; Structures and construction;
Keywords: هستند; ERT; FDEM; GPR; Embankment monitoring; Flood risk mitigation
Keywords: هستند; BPI; Brief Pain Inventory; Cre; creatinine; ERT; enzyme replacement therapy; EF; ejection fraction; eGFR; estimated glomerular filtration rate; GSL; glycosphingolipids; Gb3; globotriaosyl ceramide; LVMI; left ventricular mass index; MRI; magnetic resonanc
Keywords: هستند; CS; chondroitin sulfate; DBS; dried blood spots; DS; dermatan sulfate; ERT; enzyme-replacement therapy; GAG(s); glycosaminoglycan(s); HS; heparan sulfate; HSCT; hematopoietic stem cell transplantation; KS; cheratan sulfate; MPS; mucopolysaccharidoses; 0s;
Keywords: هستند; CNS; Central nervous system; CsA; Cyclosporine; ELC; Early Learning Composite; ERT; Enzyme replacement therapy; HCT; Hematopoietic cell transplantation; IV; Intravenous; MPS; Mucopolysaccharidosis;
Keywords: هستند; CESD; cholesteryl ester storage disease; LAL; lysosomal acid lipase; CE; cholesteryl ester; TG; triglyceride; ERT; enzyme replacement therapy; NAFLD; non-alcoholic fatty liver disease; Wolman disease; Cholesteryl ester storage disease; 1H MR spectroscopy;
Keywords: هستند; ERT; Sensitivity field; Capacitively coupled contactless conductivity detection; Optimal design;
Keywords: هستند; Ionic liquids; Gas holdup; ERT; CO2 absorption and bubble column;
Keywords: هستند; ERT; RCPT; Constrained inversion; Quick clay; Case histories
Keywords: هستند; 5â²UTR; 5â² untranslated region between the LTR and gag; A; Ala; BCFU; blue cell focus units; CA; capsid; cTAR; complementary trans-activation response DNA element; dsDNA; double-stranded DNA; ERT; endogenous reverse transcription; F16; Phe16; FA; fluor
Keywords: هستند; AD; Alzheimer's disease; Aβ; β amyloid peptide; ERT; estrogen replacement therapy; ESR; estrogen receptor; E1; estrone; E2; estradiol; E3; estriol; CYP; cytochrome P450; CYP11A1; cytochrome P450 cholesterol side chain cleavage enzyme; HSD-3β; 3β-hydro
Keywords: هستند; Rock avalanche; Rock glacier; TLS (LiDAR); Morphometry; ERT; Lichenometry; Schmidt hammer; Hornsund; Svalbard
Keywords: هستند; Integrated geophysics; Faulting; ERT; SRT; GPR; Magnetics
Use of electrical resistance tomography (ERT) for the detection of biofilm disruption mediated by biosurfactants
Keywords: هستند; ERT; Tomography; Sophorolipids; Rhamnolipids; Biofilm; Bacillus;
Enzyme replacement therapy reverses B lymphocyte and dendritic cell dysregulations in patients with Gaucher Disease
Keywords: هستند; ACE; angiotensin conversion enzyme; CBC; complete blood count; CHITO; chitotriosidase; DC; dendritic cells; ERT; enzyme replacement therapy; GCase; Glucocerebrosidase; HC; healthy control; Hgb; hemoglobin; Ig; Immunoglobulin; MGUS; monoclonal gammopathy o
Long-term outcomes with agalsidase alfa enzyme replacement therapy: Analysis using deconstructed composite events
Keywords: هستند; eGFR; estimated glomerular filtration rate; ERT; enzyme replacement therapy; FOS; Fabry Outcome Survey; LVH; left ventricular hypertrophy; NE; not estimable; Fabry disease; Enzyme replacement therapy; Agalsidase alfa; Long-term effectiveness;
Monocultures of primary porcine brain capillary endothelial cells: Still a functional in vitro model for the blood-brain-barrier
Keywords: هستند; Blood brain barrier; In vitro models; Drug delivery; Nanoparticles; Enzyme replacement therapy; Impedance spectroscopy; ABC; ATP binding casette; ASA; Arylsulfatase A; ATP; Adenosintriphosphate; Apo-E; Apolipoprotein E; AuNP; Gold nanoparticle; PBCA-NP; p
Enzyme-replacement therapy in perinatal hypophosphatasia: Case report and review of the literature
Keywords: هستند; Perinatal hypophosphatasia; Asfotase alfa; Respiratory insufficiency; Craniosynostosis; Nephrocalcinosis; Bone hypomineralization; CPAP; Continuous positive airway pressure; ERT; Enzyme-replacement therapy; HPP; Hypophosphatasia; TNSAL; tissue-nonspecific
Characteristics of 26 patients with type 3 Gaucher disease: A descriptive analysis from the Gaucher Outcome Survey
Keywords: هستند; ERT; enzyme replacement therapy; GD; Gaucher disease; GD1; type 1 Gaucher disease; GD2; type 2 Gaucher disease; GD3; type 3 Gaucher disease; GOS; Gaucher Outcome Survey; IQR; interquartile range; MN; multiples of normal; SRT; substrate reduction therapy;
Neurophysiology of hearing in patients with mucopolysaccharidosis type IV
Keywords: هستند; Mucopolysaccharidosis IV; Auditory system; Electrophysiology; Audiological testing; Hearing impairment/loss; MPS; mucopolysaccharidosis; ABR; auditory brainstem response; DPOAE; distortion products of otoacoustic emission; MEMR; middle ear muscle reflex;
Twelve years of experience with miglustat in the treatment of type 1 Gaucher disease: The Spanish ZAGAL project
Keywords: هستند; BUA; broadband ultrasound attenuation; BMD; bone mineral density; CCL18/PARC; chemokine (C-C motif) ligand 18/pulmonary and activation-regulated chemokine; CHITO; chitotriosidase; ERT; enzyme replacement therapy; DXA; dual X-ray absorptiometry; EMG; elect
Impact of loss of SOAT2 function on disease progression in the lysosomal acid lipase-deficient mouse
Keywords: هستند; CESD; cholesteryl ester storage disease; DKO; double knockout; EC; esterified cholesterol; ERT; enzyme replacement therapy; LAL; lysosomal acid lipase; LCAT; lecithin:cholesterol transferase; SOAT1; sterol O-acyltransferase 1; SOAT2; sterol O-acyltransfer
Brain white matter microstructural alterations in children of type I Gaucher disease characterized with diffusion tensor MR imaging
Keywords: هستند; WM; white matter; Type I GD; type I Gaucher disease; TD; typical development; DTI; diffusion tensor Imaging; FA; fractional anisotropy; MD; mean diffusivity; AD; Axial diffusivity; RD; radial diffusivity; GBA; beta-glucocerebrosidase; ERT; enzyme replacem
Safety, immunogenicity, and clinical outcomes in patients with Morquio A syndrome participating in 2 sequential open-label studies of elosulfase alfa enzyme replacement therapy (MOR-002/MOR-100), representing 5â¯years of treatment
Keywords: هستند; Morquio A; Mucopolysaccharidosis IVA; Long-term extension; Elosulfase alfa; 3MSCT; 3-min stair climb test; 6MWT; 6-min walk test; AE; adverse event; AUC0ât; area under the curve from time 0 to last measurable concentration; AUCinf; area under the curve
Enzyme replacement therapy with velmanase alfa (human recombinant alpha-mannosidase): Novel global treatment response model and outcomes in patients with alpha-mannosidosis
Keywords: هستند; Alpha-mannosidosis; Velmanase alfa; Enzyme-replacement therapy; Global treatment response model; OMIM 248500; Enzyme Commission number: 3.2.1.24; 3MSCT; 3-min stair climb test; 6MWT; 6-min walking test; BMI; body mass index; BOT-2; Bruininks-Oseretsky Tes
Process intensification in a chaotic SMX static mixer to achieve an energy-efficient mixing operation of non-newtonian fluids
Keywords: هستند; 2D; two-dimensions; 3D; three-dimensions; DAS; data acquisition system; ERT; electrical resistance tomography; CFD; computational fluid dynamics; SIMPLE; semi-implicit method for pressure-linked equation; PRESTO; pressure staggering option; HPCVL; high pe
Generation of osteoclasts from type 1 Gaucher patients and correlation with clinical and genetic features of disease
Keywords: هستند; GD1; Gaucher disease type 1; AVN; avascular necrosis; BMD; bone mineral density; GD; Gaucher disease; ERT; enzyme replacement therapy; SRT; substrate reduction therapy; DEXA; dual energy X-ray absorptiometry; CCL18; chemokine (C-C motif) ligand 18; MIP1-Î
'Illuminating' the interior of Kukulkan's Pyramid, Chichén Itzá, Mexico, by means of a non-conventional ERT geophysical survey
Keywords: هستند; ERT; Pyramid of Kukulkan; Constructive phases; Chichén Itzá; Mexico;
Efficacy of Idursulfase therapy in patients with Mucopolysaccharidosis type II who initiated enzyme replacement therapy in adult age. A systematic review of the literature
Keywords: هستند; Mucopolysaccharidosis type II; Enzyme replacement therapy; ERT; Idursulfase; Glycosaminoglycans; Adult; Systematic review;
Improvement of 2D ERT measurements conducted along a small earth-filled dyke using 3D topographic data and 3D computation of geometric factors
Keywords: هستند; Earth-filled dyke; ERT; Geometric factor; Numerical simulation; Monitoring;
Optimisation of electrode dimensions of ERT for non-invasive measurement applied for static liquid-gas regime identification
Keywords: هستند; non-invasive; ERT; COMSOL; Electrode;
The utility of rock-bolts as long electrodes for underground ERT surveys in mine settings
Keywords: هستند; ERT; Electrical resistivity tomography; Expansive rock-bolts; Copper mine;
Hunter syndrome: Long-term idursulfase treatment does not protect patients against DNA oxidation and cytogenetic damage
Keywords: هستند; 8-OHdG; 8-hydroxy-2â²-deoxyguanosine; 8-OHG; 8-hydroxyguanosine; 8-OHGn; 8-hydroxyguanine; BMCyt; buccal micronucleus cytome; BUD; nuclear bud; DI; damage index; ERT; enzyme replacement therapy; GAG; glycosaminoglycan; LSD; lysosmal storage disorders; MP
Using 2-D electrical resistivity imaging for joint geophysical and geotechnical characterization of shallow landslides
Keywords: هستند; Geophysics; Electrical resistivity; ERT; Soil mechanics; Landslide; Shear strength;