دانلود مقالات ISI درباره Slos + ترجمه فارسی

Propagation rate constants for the peroxidation of sterols on the biosynthetic pathway to cholesterol

Keywords: 7-DHC; 7-dehydrocholesterol; 8-DHC; 8-dehydrocholesterol; SLOS; Smith-Lemli-Opitz syndrome; CDPX2; X-linked dominant chondrodysplasia punctata; DHCR7; 7-dehydro-cholesterol reductase; MeOAMVN; 2,2′-azobis(4-methoxy-2,4-dimethylvaleronitrile); HPLC; high

دانلود رایگان متن کامل مقاله ISI 8 صفحه سال انتشار : 2017 سفارش ترجمه

تأثیر درمان دارویی روانگردان بر متابولیسم استرول

Effect of psychotropic drug treatment on sterol metabolism

Keywords: DHCR7; 7-dehydrocholesterol reductase; 7DHC; 7-dehydrocholesterol; SLOS; Smith-Lemli-Opitz syndrome; dhVitD; 1,25-dihydroxyvitamin D; 8DHC; 8-dehydrocholesterol; BHT; butylated hydroxytoluene; TPP; triphenylphosphine; PTAD; 4-phenyl-1,2,4-triazoline-3,5-d

دانلود رایگان متن کامل مقاله ISI 8 صفحه سال انتشار : 2017 سفارش ترجمه

پروب برای تزریق پروتئین به اختلالات بیوسنتز کلسترول: آلکینیل لانسترول به عنوان یک پیش ساز استرول زنده

Probes for protein adduction in cholesterol biosynthesis disorders: Alkynyl lanosterol as a viable sterol precursor

Keywords: 7-DHC; 7-dehydrocholesterol; Lath; lathosterol; Lan; lanosterol; Chol; cholesterol; SLOS; Smith-Lemli-Opitz syndrome; DHCR7; 7-dehydrocholesterol reductase; MS; mass spectrometry; APCI; atmospheric pressure chemical ionization; HPLC-; high pressure liquid

دانلود رایگان متن کامل مقاله ISI 9 صفحه سال انتشار : 2017 سفارش ترجمه

چشم انداز آینده: متابولومیکس در پزشکی آزمایشگاهی برای خطاهای متولد شده متابولیسم

The future perspective: metabolomics in laboratory medicine for inborn errors of metabolism

Keywords: GC-MS; gas chromatography mass spectrometry; LC-MS; liquid chromatography mass spectrometry; CSF; Cerebrospinal fluid; IEM; inborn errors of metabolism; MALDI; matrix-assisted laser desorption ionization; SELDI; Surface-enhanced laser desorption/ionizatio

دانلود رایگان متن کامل مقاله ISI 23 صفحه سال انتشار : 2017 سفارش ترجمه

تغذیه ناشی از استرولهای پلاسما در سندرم اسمیت-لملی-اپیتس

Feeding Impairments Associated with Plasma Sterols in Smith-Lemli-Opitz Syndrome

Keywords: 7DHC; 7-dehydrocholesterol; FTT; Failure to thrive; SLOS; Smith-Lemli-Opitz syndrome;

دانلود رایگان متن کامل مقاله ISI 7 صفحه سال انتشار : 2014 سفارش ترجمه

چگونه استرول شیب تنظیم خواص و سازمان غشاهای چربی و درج غشاء

How sterol tilt regulates properties and organization of lipid membranes and membrane insertions

Keywords: Cholesterol orientation; Lipid tilt modulus; Transitions in liquid crystals; 7DHC; SLOS; Dynorphin; Molecular dynamics simulations;

دانلود رایگان متن کامل مقاله ISI 11 صفحه سال انتشار : 2013 سفارش ترجمه

تکنیک های جرم طیف سنجی در بررسی متابولیت های استروئید به عنوان نشانگرهای بالقوه بیماری: بررسی

Mass spectrometry techniques in the survey of steroid metabolites as potential disease biomarkers: A review

Keywords: APCI-MS; Atmospheric-pressure chemical ionization mass spectrometry; APPI-MS; Atmospheric-pressure photoionization mass spectrometry; CA; Cholic acid; CAT; 1,2-Dihydroxybenzene (benzene catechol); CAT-Q; 1,2-Dihydroxybenzene-Quinone; CID; Collision-in

دانلود رایگان متن کامل مقاله ISI 12 صفحه سال انتشار : 2013 سفارش ترجمه

Dichlorophenyl piperazines, including a recently-approved atypical antipsychotic, are potent inhibitors of DHCR7, the last enzyme in cholesterol biosynthesis

Keywords: Antipyschotics; Teratogen; Cholesterol biosynthesis; Neurodevelopment; DHCR7; dehydrocholesterol reductase 7; DHCR24; dehydrocholesterol reductase 24; 7-DHC7; dehydrocholesterol; 8-DHC8; dehydrocholesterol; Des; desmosterol; Lan; lanosterol; Chol; cholest

دانلود رایگان متن کامل مقاله ISI 8 صفحه سال انتشار : 2018 سفارش ترجمه

A rare case of sterol-C4-methyl oxidase deficiency in a young Italian male: Biochemical and molecular characterization

Keywords: CDPX2; X-linked dominant chondrodysplasia punctata-2; CHILD; congenital hemidysplasia with ichthyosiform erythroderma and limb defects; dHPLC; denaturing high-performance liquid chromatography; FF-MAS; follicular fluid-meiosis activating sterol; GC-FID; g

دانلود رایگان متن کامل مقاله ISI 7 صفحه سال انتشار : 2017 سفارش ترجمه

Charge-tagging liquid chromatography-mass spectrometry methodology targeting oxysterol diastereoisomers

Keywords: ACOX2; acyl-coenzyme A oxidase 2; AMACR; alphamethylacyl-CoA racemase; CTX; cerebrotendinous xanthomatosis; CYP; cytochrome P450; EADSA; enzyme-assisted derivatisation for steroid analysis; ESI; electrospray ionisation; GC; gas chromatography; GP; Girard

دانلود رایگان متن کامل مقاله ISI 23 صفحه سال انتشار : 2017 سفارش ترجمه

MicroRNAs in brain cholesterol metabolism and their implications for Alzheimer's disease

Keywords: AAV; adeno associated virus; ABC; ATP-binding cassette; ABCA1; ABC transporter A1; AD; Alzheimer's disease; APOA-I; apolipoprotein A1; APOE; apolipoprotein E; APP; amyloid β precursor protein; BBB; blood-brain barrier; CE; cholesteryl ester; CETP; chol

دانلود رایگان متن کامل مقاله ISI 9 صفحه سال انتشار : 2016 سفارش ترجمه

Chemical derivatization for enhancing sensitivity during LC/ESI-MS/MS quantification of steroids in biological samples: a review

Keywords: 1,25(OH)2D2; 1α,25-dihydroxyvitamin D2; 1,25(OH)2D3; 1α,25-dihydroxyvitamin D3; 17(OH)P4; 17α-hydroxyprogesterone; 17(OH)P5; 17α-hydroxypregnenolone; 24,25(OH)2D3; 24R,25-dihydroxyvitamin D3; 25(OH)D2; 25-hydroxyvitamin D2; 25(OH)D3; 25-hydroxyvitamin

دانلود رایگان متن کامل مقاله ISI 13 صفحه سال انتشار : 2016 سفارش ترجمه

DHCR7: A vital enzyme switch between cholesterol and vitamin D production

Keywords: 7DHC; 7-dehydrocholesterol; AEBS; antiestrogen binding site; DHCR7; 7-dehydrocholesterol reductase; DHCR14; Δ14-sterol reductase; DHCR24; 24-dehydrocholesterol reductase; EBP; emopamil binding protein; Hh; Hedgehog; HMGCR; 3-hydroxy-3-methylglutaryl-CoA

دانلود رایگان متن کامل مقاله ISI 46 صفحه سال انتشار : 2016 سفارش ترجمه

Bridging NCL research gaps

Keywords: NCL; neuronal ceroid lipofuscinosis; LSD; lysosomal storage disease; ERT; enzyme replacement therapy; ER; endoplasmic reticulum; PoC; proof-of-concept; iPS cell; induced pluripotent stem cell; PD; Parkinson's disease; NPC; Niemann-Pick type C; SLOS; Smi

دانلود رایگان متن کامل مقاله ISI 5 صفحه سال انتشار : 2015 سفارش ترجمه

The sterol-based transcriptional control of human 7-dehydrocholesterol reductase (DHCR7): Evidence of a cooperative regulatory program in cholesterol synthesis

Keywords: 7DHC; 7-dehydrocholesterol; DHCR7; 7-dehydrocholesterol reductase; DHCR24; 24-dehydrocholesterol reductase; LDLR; low-density lipoprotein receptor; NF-Y; nuclear factor-Y; NPC1L1; Niemann-pick C1 like-1; SLOS; Smith-Lemli-Opitz Syndrome; Sp1; specific

دانلود رایگان متن کامل مقاله ISI 9 صفحه سال انتشار : 2014 سفارش ترجمه

No. 261-Prenatal Screening for Fetal Aneuploidy in Singleton Pregnancies

Keywords: Aneuploidy; Down syndrome; trisomy; prenatal screening; genetic health risk; genetic health surveillance; prenatal diagnosis; AFP; alpha fetoprotein; CVS; chorionic villus sampling; DR; detection rate; FPR; false-positive rate; FTS; first trimester screen

دانلود رایگان متن کامل مقاله ISI 15 صفحه سال انتشار : 2017 سفارش ترجمه

A routine method for cholesterol and 7-dehydrocholesterol analysis in dried blood spot by GC–FID to diagnose the Smith–Lemli–Opitz syndrome

Keywords: DBS; GC–FID; SLOS

دانلود رایگان متن کامل مقاله ISI 5 صفحه سال انتشار : 2012 سفارش ترجمه

Photosensitivity disorders in children

Keywords: children; double-strand break DNA repair; hereditary; nucleotide excision repair; photoaggravated; photodermatoses; photosensitivity; phototesting; AD; atopic dermatitis; BS; Bloom syndrome; COFS; cerebro-oculo-facio-skeletal syndrome; CS; Cockayne syndro

دانلود رایگان متن کامل مقاله ISI 15 صفحه سال انتشار : 2012 سفارش ترجمه

Synthesis and photochemical transformation of 3β,21-dihydroxypregna-5,7-dien-20-one to novel secosteroids that show anti-melanoma activity

Keywords: Secosteroids; UV radiation; Vitamin D; SLOS; Skin; Melanoma;

دانلود رایگان متن کامل مقاله ISI 11 صفحه سال انتشار : 2011 سفارش ترجمه

Plasma Plant Sterol Levels Do Not Reflect Cholesterol Absorption in Children with Smith-Lemli-Opitz Syndrome

Keywords: GC; Gas chromatography; GCMS; Gas chromatography mass spectrometry; OHSU; Oregon Health & Science University; SLOS; Smith-Lemli-Opitz syndrome;

دانلود رایگان متن کامل مقاله ISI 6 صفحه سال انتشار : 2009 سفارش ترجمه

Steroid production and excretion by the pregnant mouse, particularly in relation to pregnancies with fetuses deficient in Δ7-sterol reductase (Dhcr7), the enzyme associated with Smith–Lemli–Opitz syndrome

Keywords: SLOS; Smith–Lemli–Opitz syndrome; Mouse pregnancy steroids; GC/MS; DHCR7

دانلود رایگان متن کامل مقاله ISI 10 صفحه سال انتشار : 2009 سفارش ترجمه

Photo-conversion of two epimers (20R and 20S) of pregna-5,7-diene-3β, 17α, 20-triol and their bioactivity in melanoma cells

Keywords: Secosteroids; UV radiation; Lumisterol; Tachysterol; SLOS; Melanoma

دانلود رایگان متن کامل مقاله ISI 11 صفحه سال انتشار : 2009 سفارش ترجمه

Cholesterol biosynthesis from birth to adulthood in a mouse model for 7-dehydrosterol reductase deficiency (Smith–Lemli–Opitz syndrome)

Keywords: SLOS; Cholesterol deficiency; Dehydrocholesterol; Mutant mouse model; Sterol metabolism; Dhcr7

دانلود رایگان متن کامل مقاله ISI 7 صفحه سال انتشار : 2007 سفارش ترجمه

Inability to fully suppress sterol synthesis rates with exogenous sterol in embryonic and extraembyronic fetal tissues

Keywords: SLOS; Smith-Lemli-Opitz syndrome; DHCR7; 3β-hydroxysterol Δ7-reductase; HMGR; HMG-CoA reductase; SREBP; sterol regulatory element binding protein; SCAP; SREBP cleavage-activating protein; DPS; digitonin-precipitable sterols; Shh; Sonic hedgehog; Cho

دانلود رایگان متن کامل مقاله ISI 8 صفحه سال انتشار : 2007 سفارش ترجمه

SLOS carrier frequency in Poland as determined by screening for Trp151X and Val326Leu DHCR7 mutations

Keywords: SLOS; Carrier frequency; DHCR7 mutations; Polish population

دانلود رایگان متن کامل مقاله ISI 6 صفحه سال انتشار : 2006 سفارش ترجمه

Yolk sac cholesteryl ester secretion rates can be manipulated in the Golden Syrian hamster: Effect of yolk sac cholesterol concentrations

Keywords: Smith-Lemli-Opitz syndrome; SLOS; 3β-hydroxysteroid-delta7-reductase; Δ7 reductase; apolipoprotein; apo; Eagle's Basal Media; EBM; lactate dehydrogenase; LDH; Sonic hedgehog; Shh; Fetus; Lipoprotein; Smith-Lemli-Opitz syndrome; Visceral endoderm

دانلود رایگان متن کامل مقاله ISI 8 صفحه سال انتشار : 2005 سفارش ترجمه

The teratology of autism

Keywords: VPA; valproic acid; ASD; autism spectrum disorder; SLOS; Smith-Lemli-Opitz syndrome; RARE; retinoic acid responsive element; RA; retinoic acid; CS; conditioned stimulus; US; unconditioned stimulus; CR; conditioned response; UR; unconditioned response;

دانلود رایگان متن کامل مقاله ISI 11 صفحه سال انتشار : 2005 سفارش ترجمه

Residual cholesterol synthesis and simvastatin induction of cholesterol synthesis in Smith-Lemli-Opitz syndrome fibroblasts

Keywords: Smith-Lemli-Opitz syndrome; SLOS; 7-Dehydrocholesterol reductase; DHCR7; Cholesterol synthesis; Simvastatin;

دانلود رایگان متن کامل مقاله ISI 12 صفحه سال انتشار : 2005 سفارش ترجمه