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Elsevier电子期刊(30)

在“Elsevier电子期刊”中，命中：30条，耗时：小于0.01 秒

在所有数据库中总计命中：30条

1.Differences in cleavage of globotriaosylceramide and its derivatives accumulated in organs of young Fabry mice following enzyme replacement therapy

作者：Takashi Kodama^a ; Takahiro Tsukimura^a ; Ikuo Kawashima^b ; Atsuko Sato^c ; Hitoshi Sakuraba^c ; Tadayasu Togawa^a ; ^{tadayasu@my-pharm.ac.jp}

关键词：Fabry disease ; α-Galactosidase A ; α-Galactosidase A knockout mouse ; Globotriaosylceramide ; Globotriaosylsphingosine ; Enzyme replacement therapy

刊名：Molecular Genetics and Metabolism

出版年：2017

2.Plasma LysoGb3: A useful biomarker for the diagnosis and treatment of Fabry disease heterozygotes

作者：Albina Nowak^a ; ^{albina.nowak@usz.ch} ; Thomas P. Mechtler^b ; ^{t.mechtler@archimedlife.com} ; Robert J. Desnick^c ; ^{robert.desnick@mssm.edu} ; David C. Kasper^b ; ^{d.kasper@archimedlife.com}

关键词：Fabry disease ; Heterozygotes ; LysoGb3 ; Biomarker ; α-galactosidase deficiency

刊名：Molecular Genetics and Metabolism

出版年：2017

3.Molecular and clinical studies in five index cases with novel mutations in the GLA gene

作者：Carmela Zizzo^a ; ¹ ; ^{zizzo@ibim.cnr.it" class="auth_mail" title="E-mail the corresponding author} ; Ines Monte^b ; ¹ ; Antonio Pisani^c ; ¹ ; Pasquale Fatuzzo^d ; Eleonora Riccio^c ; Margherita Stefania Rodolico^e ; Paolo Colomba^a ; Maurizio Uva^f ; Giuseppe Cammarata^a ; Riccardo Alessandro^a ; ^g ; Francesco Iemolo^a ; Giovanni Duro^a

关键词：FD ; Fabry disease ; α-GAL A ; α-galactosidase A ; GB3 ; globotriaosylceramide ; Lyso-GB3 ; globotriaosylsphingosine ; GLA ; GLA gene ; ND ; not determined ; DGJ ; 1-deoxygalactonojirimycin ; ISE ; intron splicing enhancer

刊名：Gene

出版年：2016

4.Development of a model system for neuronal dysfunction in Fabry disease

作者：Christine R. Kaneski^a ; ^{kaneskic@ninds.nih.gov" class="auth_mail" title="E-mail the corresponding author} ; Roscoe O. Brady^a ; John A. Hanover^b ; Ulrike H. Schueler^b

关键词：4-MU ; 4-methylumbelliferone ; AGA ; alpha-galactosidase A ; ACh ; acetylcholine ; B2M ; beta-2-microglobulin ; CTNF ; ciliary neurotrophic factor ; FACS ; fluorescence activated cells sorting ; HEX ; hexosaminidase ; HRP ; horseradish peroxidase ; Gb3 ; globotriaosylceramide ; GCS ; GemCell&trade ; Calf Serum ; lyso-Gb3 ; globotriaosylsphingosine ; pChAT ; peripheral acetylcholine transferase ; qRT-PCR ; quantitative real-time reverse-transcription polymerase chain reaction ; RFU ; relative fluorescence units ; shRNA ; shor

刊名：Molecular Genetics and Metabolism

出版年：2016

5.Biomolecules damage and redox status abnormalities in Fabry patients before and during enzyme replacement therapy

作者：Giovana Brondani Biancini^a ; ^b ; ^{giovana.bb@gmail.com" class="auth_mail" title="E-mail the corresponding author} ; Carlos Eduardo Jacques^b ; ^c ; Tatiane Hammerschmidt^b ; Heryk Motta de Souza^b ; Bruna Donida^a ; ^b ; Marion Deon^b ; Filippo Pinto Vairo^b ; Charles Marques Lourenç ; o^d ; Roberto Giugliani^a ; ^b ; ^e ; Carmen Regla Vargas^a ; ^b ; ^c ; ^{crvargas@hcpa.edu.br" class="auth_mail" title="E-mail the corresponding author}

关键词：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 ; reduced glutathione ; GSSG ; oxidized glutathione ; H2O2 ; hydrogen peroxide ; Lyso-Gb3 ; globotriaosylsphingosine ; MDA ; malondialdehyde ; NO3&minus ; ; nitrate ; NO. ; nitric oxide ; NO2&minus ; ; nitrite ; ONOO&minus ; ; peroxinytrite ; RNS ; rea

刊名：Clinica Chimica Acta

出版年：2016

6.Lyso-glycosphingolipid abnormalities in different murine models of lysosomal storage disorders

作者：Maria J. Ferraz^a ; ¹ ; André ; R.A. Marques^a ; ¹ ; Paulo Gaspar^b ; ^c ; ^d ; ¹ ; Mina Mirzaian^e ; ¹ ; Cindy van Roomen^a ; Roelof Ottenhoff^a ; Pilar Alfonso^f ; Pilar Irú ; n^f ; Pilar Giraldo^f ; Patrick Wisse^g ; Clara Sá ; Miranda^b ; ^c ; Herman S. Overkleeft^g ; Johannes M. Aerts^a ; ^e ; ^{j.m.f.g.aerts@lic.leidenuniv.nl" class="auth_mail" title="E-mail the corresponding author}

关键词：GSL ; glycosphingolipid ; Lyso-GSL ; glycosphingoid base ; Lyso-Gb3 ; globotriaosylsphingosine ; GlcSph ; glucosylsphingosine ; GlySph ; glycosylsphingosine ; GlyCer ; glycosylceramide

刊名：Molecular Genetics and Metabolism

出版年：2016

7.Tandem mass spectrometry multiplex analysis of methylated and non-methylated urinary Gb₃ isoforms in Fabry disease patients

作者：Mona Abaoui ; Michel Boutin ; Pamela Lavoie ; Christiane Auray-Blais ; ^{Christiane.auray-blais@usherbrooke.ca" class="auth_mail" title="E-mail the corresponding author}

关键词：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 dihexoside ; lyso-Gb3 ; globotriaosylsphingosine ; REB ; Research Ethics Board ; CHUS ; Centre hospitalier universitaire de Sherbrooke ; MeOH ; methanol ; MTBE ; methyl tert-butyl ether ; IS ; internal standard ; ESI + ; ; positive electrospray ionization ; M

刊名：Clinica Chimica Acta

出版年：2016

8.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

作者：Mina Mirzaian^a ; Patrick Wisse^b ; Maria J. Ferraz^c ; André ; R.A. Marques^c ; Tanit L. Gabriel^c ; Cindy P.A.A. van Roomen^c ; Roelof Ottenhoff^c ; Marco van Eijk^a ; Jeroen D.C. Codé ; e^b ; Gijsbert A. van der Marel^b ; Herman S. Overkleeft^b ; Johannes M. Aerts^a ; ^{j.m.f.g.aerts@lic.leidenuniv.nl" class="auth_mail" title="E-mail the corresponding author}

关键词：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

刊名：Clinica Chimica Acta

出版年：2016

9.Globotriaosylsphingosine (lyso-GB3) as useful marker for monitoring initial therapeutic outcomes of enzyme replacement therapy for patients with Fabry disease

作者：Elena Verrecchia ; Maria Giovinale ; Alessandra Paolucci ; Daniela Antuzzi ; Raffaele Manna

刊名：Molecular Genetics and Metabolism

出版年：2016

10.Evaluation of Proinflammatory Prognostic Biomarkers for Fabry Cardiomyopathy With Enzyme Replacement Therapy

作者：Kuan-Hsuan Chen ; RPh^a ; Yueh Chien ; PhD^b ; ^g ; ⁱ ; Kang-Ling Wang ; MD^c ; ^f ; Hsin-Bang Leu ; MD^a ; ^e ; ^f ; Chen-Yuan Hsiao ; MD^d ; Ying-Hsiu Lai ; PhD^g ; Chien-Ying Wang ; MD^c ; ^g ; Yuh-Lih Chang ; PhD^b ; ^g ; Shing-Jong Lin ; MD^a ; ^f ; ^g ; Dau-Ming Niu ; MD^a ; ^h ; Shih-Hwa Chiou ; MD^a ; ^b ; ^g ; Wen-Chung Yu ; MD^c ; ^f ; ^{wcyu@vghtpe.gov.tw" class="auth_mail" title="E-mail the corresponding author}

刊名：Canadian Journal of Cardiology

出版年：2016

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