Keywords: سیستینوز; Proximal renal tubular acidosis; Fanconi syndrome; Cystinosis; Lowe Syndrome; Dent disease;
مقالات ISI سیستینوز (ترجمه نشده)
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در صورتی که به ترجمه آماده هر یک از مقالات زیر نیاز داشته باشید، می توانید سفارش دهید تا مترجمان با تجربه این مجموعه در اسرع وقت آن را برای شما ترجمه نمایند.
Keywords: سیستینوز; Cystinosis; Cornea; Crystallization; Collagen;
Keywords: سیستینوز; Bartter's; cystinosis; diabetes insipidus; Fanconi; Gitelman's; polydipsia; polyuria; pseudohypoaldosteronism; renal tubular acidosis; renal tubular disorder; tubulopathy;
Keywords: سیستینوز; Cysteamine; Cystinosis; Eye drop formulations; Stability; Oxidation; Vitamin E; OMAC;
Keywords: سیستینوز; Cystinosis; Distal myopathy; Slow myosin; CTNS;
Keywords: سیستینوز; Cystinosis; Cysteamine; Halitosis; Carbohydrate-cysteamine thiazolidines; Pro-drug; L-Cys-T; lactose-cysteamine thiazolidine; R-Cys-T; ribose-cysteamine thiazolidine; A-Cys-T; arabinose-cysteamine thiazolidine; G-Cys-T; glucose-cysteamine thiazolidine; M-
Keywords: سیستینوز; Cystinosis; Ophthalmic delivery; Gel; Cysteamine; Preformulation;
Keywords: سیستینوز; Transición; Adolescente; Cistinosis; Trasplante renal; Adherencia; Transition; Adolescent; Cystinosis; Kidney transplant; Adherence;
Keywords: سیستینوز; Transition; Adolescent; Cystinosis; Kidney transplant; Adherence; Transición; Adolescente; Cistinosis; Trasplante renal; Adherencia;
Keywords: سیستینوز; cysteamine; cystinosis; Fabry's disease; Fanconi's syndrome; kidney failure; kidney transplantation; methylmalonic aciduria; primary hyperoxaluria;
Keywords: سیستینوز; Cystinosis; Cysteamine; Fanconi syndrome; White blood cell (WBC) cystine levels; Transition; AdherenceCistinosis; Cisteamina; Síndrome de Fanconi; Cistina intraleucocitaria; Transición; Adherencia
Keywords: سیستینوز; Bartter's; cystinosis; diabetes insipidus; Fanconi; Gitelman's; polydipsia; polyuria; pseudohypoaldosteronism; renal tubular acidosis; renal tubular disorder; tubulopathy
Keywords: سیستینوز; BV; Baculovirus, also used as emptyBac (no transgene insert); sialBac; (BV containing the human sialin sequence); cysBac; (BV containing the human CTNS sequence); BVES; Baculovirus Expression System; Sf9; Spodptera frugiperda cell line 9 (from ovarian tis
In vitro drug release and in vivo safety of vitamin E and cysteamine loaded contact lenses
Keywords: سیستینوز; Cystinosis; Cysteamine; Vitamin E; Contact lenses; Drug delivery; Drug degradation; Modeling;
Effect of pH and penetration enhancers on cysteamine stability and trans-corneal transport
Keywords: سیستینوز; Ocular delivery; Trans-corneal delivery; Cysteamine; Stability; Eye-drop; Cyclodextrin; Benzalkonium; Cystinosis; EDTA
Activation of the transcription factor EB rescues lysosomal abnormalities in cystinotic kidney cells
Keywords: سیستینوز; cystinosis; pediatric nephrology; proximal tubule; renal pathology;
CTNS mutations in publicly-available human cystinosis cell lines
Keywords: سیستینوز; CTNS; Sequencing; Cystinosis; Mutation; Splicing; Coriell Cell Repository
The hen’s egg chorioallantoic membrane (HET-CAM) test to predict the ophthalmic irritation potential of a cysteamine-containing gel: Quantification using Photoshop® and ImageJ
Keywords: سیستینوز; HET-CAM test; ImageJ; Photoshop®; Ophthalmic; Cystinosis; Cysteamine
A new gel formulation of topical cysteamine for the treatment of corneal cystine crystals in cystinosis: The Cystadrops OCT-1 study
Keywords: سیستینوز; Cystinosis; Cornea; Cysteamine hydrochloride; Gel formulation
LC-MS/MS measurement of leukocyte cystine; effect of preanalytic factors
Keywords: سیستینوز; Leukocyte cystine; LC-MS/MS; Cystinosis; Preanalytic factors; Sample pre-treatment;
Improvement of the cystine measurement in granulocytes by liquid chromatograhy-tandem mass spectrometry
Keywords: سیستینوز; HPLC-ESI-MS/MS; Cystinosis; Cystine in granulocytes
Feasibility of corneal drug delivery of cysteamine using vitamin E modified silicone hydrogel contact lenses
Keywords: سیستینوز; Cystinosis; Contact lenses; Extended drug delivery; Vitamin E; Drug stability; Cysteamine
Discovery of functional protein groups by clustering community links and integration of ontological knowledge
Keywords: سیستینوز; Cystinosis; Protein-to-protein interactions; Clustering; Ontologies
A homemade MLPA assay detects known CTNS mutations and identifies a novel deletion in a previously unresolved cystinosis family
Keywords: سیستینوز; bp; basepairs; CTNS; cystinosin; kb; kilobases or 1000Â bp; MLPA; multiplex ligation-dependent probe amplification; PCR; polymerase chain reaction; RS-values; relative signal values; UTR; untranslated region; Copy number; CTNS; Cystinosis; Deletion; MLPA;
Studying nonobstructive azoospermia in cystinosis: histologic examination of testes and epididymis and sperm analysis in a Ctns−/− mouse model
Keywords: سیستینوز; Cystinosis; infertility; testis; epididymis; sperm analysis; CASA; nonobstructive azoospermia
Activation of AMP kinase plays a role in the increased apoptosis in the renal proximal tubule in cystinosis
Keywords: سیستینوز; Cystinosis; Renal proximal tubule; AMP kinase; Primary culture; siRNA;
Suppository formulations as a potential treatment for nephropathic cystinosis
Keywords: سیستینوز; Cystinosis; Formulation; PEG; Physical Stability; Dissolution; Thermal Analysis; Pediatric;
Cysteamine therapy delays the progression of nephropathic cystinosis in late adolescents and adults
Keywords: سیستینوز; complications; cysteamine; cystinosis
Generation of the oxidized form protects human brain type creatine kinase against cystine-induced inactivation
Keywords: سیستینوز; CK, creatine kinase; BBCK, brain type creatine kinase; MMCK, muscle type creatine kinase; O-CK, oxidized form of creatine kinase; SDS, sodium dodecyl sulfate; ANS, 1-anilinonaphtalene-8-sulfonate; CD, circular dichroismCystine; Cystinosis; Brain type crea
Synthesis and in vitro evaluation of novel pro-drugs for the treatment of nephropathic cystinosis
Keywords: سیستینوز; Cystinosis; Cysteamine; Cystamine; Pro-drugs
Reduced phosphate transport in the renal proximal tubule cells in cystinosis is due to decreased expression of transporters rather than an energy defect
Keywords: سیستینوز; Cystinosis; Renal proximal tubule; Phosphate transport; Primary culture; siRNA
Kidney preservation by bone marrow cell transplantation in hereditary nephropathy
Keywords: سیستینوز; bone marrow stem cells; chronic kidney injury; cystinosis; donor-derived blood cell engraftment;
Fertility status in male cystinosis patients treated with cysteamine
Keywords: سیستینوز; Cystinosis; infertility; azoospermia; cysteamine
The incidence of atubular glomeruli in nephropathic cystinosis renal biopsies
Keywords: سیستینوز; Cystinosis; Atubular glomerulii; Apoptosis; Swan neck deformity; Renal biopsy
The redox status of cystinotic fibroblasts
Keywords: سیستینوز; Cystinosis; Redox; Fibroblasts;
Gel formulations for treatment of the ophthalmic complications in cystinosis
Keywords: سیستینوز; Cystinosis; Ophthalmic delivery; Gel; Modified release
Renal tubular disorders
Keywords: سیستینوز; Bartter's; cystinosis; Fanconi; Gitelman's; polydipsia; polyuria; pseudohypoaldosteronism; renal tubular disorder; tubulopathy
Developmental changes in cerebral white matter microstructure in a disorder of lysosomal storage
Keywords: سیستینوز; Cystinosis; Lysosomal storage disorder; DTI; Children; Visuospatial
Cystinosis of the brain and spinal cord with associated vasculopathy
Keywords: سیستینوز; Cystinosis; Brain; Spinal cord; Vasculitis;
Visual and verbal learning in a genetic metabolic disorder
Keywords: سیستینوز; Cystinosis; Learning; Memory; CVLT; White matter; Non-verbal learning disability; Lysosomal storage disease; Visuospatial;
Molecular and cellular basis of lysosomal transmembrane protein dysfunction
Keywords: سیستینوز; Lysosomal storage disorder; Lysosomal membrane; Transporter; Channel; Cystinosis; Salla disease; Infantile sialic acid disorder; Cobalamin F type disease; Mucopolysaccharidosis; Sanfilippo C syndrome; Osteopetrosis; Mucolipidosis type IV; Danon disease; C
Cystine accumulation in the CNS results in severe age-related memory deficits
Keywords: سیستینوز; Cystinosis; Cystine transport; Lysosomal storage disorder; Learning and memory; Age-related deficits
A potential new prodrug for the treatment of cystinosis: Design, synthesis and in-vitro evaluation
Keywords: سیستینوز; Cystinosis; Prodrug; Treatment
Characterization of mammalian sedoheptulokinase and mechanism of formation of erythritol in sedoheptulokinase deficiency
Keywords: سیستینوز; Sedoheptulose; Heptose; Cystinosis; Sugar kinase; Pentose-phosphate pathway; Volemitol;
Urinary Protein Excretion Pattern and Renal Expression of Megalin and Cubilin in Nephropathic Cystinosis
Keywords: سیستینوز; Cystinosis; Fanconi syndrome; proteinuria; megalin; cubilin; low-molecular-weight proteins;
The origin of halitosis in cystinotic patients due to cysteamine treatment
Keywords: سیستینوز; Cystinosis; Halitosis; Cysteamine; Dimethylsulphide; Methanethiol
Cystine Inhibits Creatine Kinase Activity in Pig Retina
Keywords: سیستینوز; Creatine kinase; Retina; Cystine; Cysteamine; Cystinosis
Measurement of cystine in granulocytes using liquid chromatography-tandem mass spectrometry
Keywords: سیستینوز; Cystinosis; Polymorphonuclear leukocyte (PMN) cystine measurement; Tandem mass spectrometry
A review of the role of enhanced apoptosis in the pathophysiology of cystinosis
Keywords: سیستینوز; Cystinosis; Apoptosis; Cysteinylation; PKC δ; Swan neck deformity; Atubular glomeruli; Modifier genes; Cystinosis variants
Ophthalmic Manifestations and Histopathology of Infantile Nephropathic Cystinosis: Report of a Case and Review of the Literature
Keywords: سیستینوز; cystine; cystinosis; eye; histopathology; infantile nephropathic cystinosis; lysosome