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Elsevier电子期刊(35606)

在“Elsevier电子期刊”中，命中：35,606条，耗时：小于0.01 秒

在所有数据库中总计命中：35,606条

1.Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation

作者：Venkateshwar Mutyam^a ; Emily Falk Libby^d ; Ning Peng^a ; Denis Hadjiliadis^e ; Michael Bonk^e ; George M. Solomon^a ; ^d ; Steven M. Rowe^a ; ^b ; ^c ; ^d ; ^{smrowe@uab.edu}

关键词：PTC mutations ; W1282X ; CFTR potentiator ; Ivacaftor ; Readthrough

刊名：Journal of Cystic Fibrosis

出版年：2017

2.The effects of ivacaftor on CF fatty acid metabolism: An analysis from the GOAL study

作者：Michael Glenn O'Connor^a ; ^{michael.g.oconnor@vanderbilt.edu} ; Adam Seegmiller^b

关键词：Fatty acids ; Cystic fibrosis ; Ivacaftor ; Prostaglandin E

刊名：Journal of Cystic Fibrosis

出版年：2017

3.Changes of CFTR functional measurements and clinical improvements in cystic fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor

作者：Myriam Mesbahi^a ; ¹ ; Michal Shteinberg^b ; ^c ; ¹ ; Michael Wilschanski^d ; Aurelie Hatton^a ; Thao Nguyen&ndash ; Khoa^a ; Hannah Friedman^d ; Michael Cohen^d ; Virginie Escabasse^e ; Muriel Le Bourgeois^a ; Vicenzina Lucidi^f ; Isabelle Sermet-Gaudelus^f ; ^{Isabelle.sermet@aphp.fr} ; Laurence Bassinet^g ; ² ; Galit Livnat^b ; ^c ; ²

关键词：Cystic fibrosis ; Nasal potential difference ; Evaporimetry ; Short circuit current ; Ivacaftor ; CFTR modulators

刊名：Journal of Cystic Fibrosis

出版年：2017

4.Structure-activity relationships of ω-Agatoxin IVA in lipid membranes

作者：Jae Ha Ryu^a ; Hoi Jong Jung^a ; Shiro Konishi^b ; Ha Hyung Kim^c ; Zee-Yong Park^a ; ^{zeeyong@gist.ac.kr} ; Jae Il Kim^a ; ^{jikim@gist.ac.kr}

关键词：ω-Agatoxin IVA ; Gating modifier toxin ; Cav2.1

刊名：Biochemical and Biophysical Research Communications

出版年：2017

5.Mucopolysaccharidosis IVA and glycosaminoglycans

作者：Shaukat Khan^a ; ¹ ; Carlos J. Almé ; ciga-Dí ; az^b ; ¹ ; Kazuki Sawamoto^a ; William G. Mackenzie^a ; Mary C. Theroux^a ; Christian Pizarro^a ; Robert W. Mason^a ; Tadao Orii^c ; Shunji Tomatsu^a ; ^c ; ^d ; ^{stomatsu@nemours.org} ; ^{stomatsu@gifu-u.ac.jp}

关键词：MPS ; mucopolysaccharidosis ; GAG ; glycosaminoglycans ; C6S ; chondroitin-6-sulfate ; KS ; keratin sulfate ; ECM ; extracellular matrix ; GALNS ; N-acetylgalactosamine 6-sulfate sulfatase ; LC-MS/MS ; liquid chromatography/tandem mass spectrometry ; LSD ; lysosomal storage disorder ; DBS ; dried blood spot ; DMB ; dimethylmethylene blue ; ERT ; enzyme replacement therapy ; 6MWT ; 6 ; min walk test ; 3MSCT ; 3 ; min stair climb test ; ADL ; activity of daily living ; HSCT ; Hematopoietic Stem Cell Therapy ; BMT ; bone m

刊名：Molecular Genetics and Metabolism

出版年：2017

6.Correlation of sweat chloride and percent predicted FEV₁ in cystic fibrosis patients treated with ivacaftor

作者：Meredith C. Fidler^a ; ^{Meredith_fidler@vrtx.com} ; Jack Beusmans^b ; Paul Panorchan^b ; Fredrick Van Goor^a

关键词：CF ; cystic fibrosis ; CFTR ; CF transmembrane conductance regulator ; CI ; confidence interval ; ppFEV1 ; percent predicted forced expiratory volume in 1 s ; q12h ; every 12 h

刊名：Journal of Cystic Fibrosis

出版年：2017

7.Ivacaftor and symptoms of extra-oesophageal reflux in patients with cystic fibrosis and G551D mutation

作者：Gemma L. Zeybel^a ; Jeffrey P. Pearson^a ; Amaran Krishnan^c ; Stephen J. Bourke^d ; Simon Doe^d ; Alan Anderson^d ; Shoaib Faruqi^e ; Alyn H. Morice^e ; Rhys Jones^c ; Melissa McDonnell^a ; Mujdat Zeybel^b ; ^f ; Peter W. Dettmar^g ; Malcolm Brodlie^b ; ^h ; Chris Ward^b ; ^c ; ^{Chris.Ward@newcastle.ac.uk}

关键词：G551D ; CFTR potentiator ; Gastrointestinal ; Real life data

刊名：Journal of Cystic Fibrosis

出版年：2017

8.Modelo de inclusión tecnológica UAV para la prevención de trabajos de alto riesgo, en industrias de la construcción basado en la metodología IVAS

作者：Alfredo Toriz P. ; ^{alfredo.toriz@upaep.mx} ; Modesto Raygoza B. ; Daniel Martí ; nez N.

关键词：Robó ; tica de servicio ; Vehí ; culos aé ; reos no tripulados (UAV) ; inclusió ; n tecnoló ; gica

刊名：Revista Iberoamericana de Automática e Informática Industrial RIAI

出版年：2017

9.Four novel mutations in the N-acetylgalactosamine-6-sulfate sulfatase gene among Egyptian patients with Morquio A disease

作者：Ekram M. Fateen^a ; ^{efateen@yahoo.com} (Professor) ; Hanan Abd El Mawgoud^b ; ^{hananmoawad@gmail.com} (Assistant Professor) ; Noura R. Eissa^c ; ^{nr.eissa@yahoo.com} (Assistant Reseacher) ; Mona M. Ibrahim^a ; ^{monamahmoud60@yahoo.com} (Assistant Professor) ; Mona S. Aglan^d ; ^{drmona_aglan@yahoo.com} (Professor) ; Mona L. Essawi^c ; ^{mlessawi@yahoo.com} (Professor)

关键词：MPS IVA ; Mucopolysaccharidosis type 4 &ndashA ; GALNS ; N-acetylgalactosamine-6-sulfatase ; GAGs ; Glycosaminoglycans ; NRC ; National Research Centre ; MPSs ; Mucopolysaccharidoses ; GLB1 ; β-Galactosidase ; KS ; Keratan Sulfate ; C6S ; Chondroitin-6-Sulfate ; kDa ; Kilo Dalton ; HGMD ; Human Gene Mutation Database ; ERT ; Enzyme Replacement Therapy ; LMSDC ; Limb Malformation and Skeletal Deformities Clinic ; RFLP ; Restriction Fragment Length Polymorphism ; PCR ; Polymerase Chain Reaction ; mRNA ; messenger Ribonucleic

刊名：Gene

出版年：2017

10.A Case Report of Pregnancy During Use of Targeted Therapeutics for Cystic Fibrosis

作者：Sigrid Ladores ; ^{sladores@uab.edu}Author Vitae ; Traci M. KazmerskiAuthor Vitae ; Steven M. RoweAuthor Vitae

关键词：cystic fibrosis ; fertility ; ivacaftor ; lumacaftor/ivacaftor ; sexual and reproductive health

刊名：Journal of Obstetric, Gynecologic & Neonatal Nursing

出版年：2017

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