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Elsevier电子期刊(168)

在“Elsevier电子期刊”中，命中：168条，耗时：小于0.01 秒

在所有数据库中总计命中：168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.Accumulation of globotriaosylceramide (GL3) in cardiomyocytes (CM) is progressive with age and inversely correlates with baseline alpha galactosidase A (AGALA) activity in enzyme replacement therapy (ERT)-naïve Fabry patients with IVS4 + 919G >/; A mutation

作者：Fu-Pang Chang^a ; ^b ; Ting-Rong Hsu^a ; Sheng-Che Hung^a ; Shih-Hsien Sung^a ; Wen-Chung Yu^a ; Dau-Ming Niu^a ; Behzad Najafian^b

刊名：Molecular Genetics and Metabolism

出版年：2017

3.Reevaluate current routine histopathologic examinations for Fabry disease- not sensitive enough to identify early globotriaosylceramide accumulation in cardiomyocytes

作者：Dau-Ming Niu^a ; ^b ; Fu-Pang Chang^a ; Ming-Jia Hsu^b ; Chia-Lin Hsu^b ; Ting-Rong Hsu^a ; ^b ; Sheng-kai Chang^a ; Yung-Hsiu Lu^a ; ^b

刊名：Molecular Genetics and Metabolism

出版年：2017

4.Podocyte globotriaosylceramide (GL-3) content strongly impacts age-dependent podocyte loss in ERT-naïve male Fabry patients

刊名：Molecular Genetics and Metabolism

出版年：2017

5.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

6.Role of protein kinase A and class II phosphatidylinositol 3-kinase C2β in the downregulation of KCa3.1 channel synthesis and membrane surface expression by lyso-globotriaosylceramide

作者：Ju Yeon Choi ; Seonghee Park ; ^{sp@ewha.ac.kr" class="auth_mail" title="E-mail the corresponding author}

关键词：Lyso-globotriaosylceramide ; KCa3.1 channel ; Protein kinase A ; Class II phosphatidylinositol 3-kinase C2β ; Phosphatidylinositol 3-phosphate

刊名：Biochemical and Biophysical Research Communications

出版年：2016

7.Determining the Turnover of Glycosphingolipid Species by Stable-Isotope Tracer Lipidomics

作者：Tore Skotland¹ ; ² ; ^&dagger ; ; ^{Tore.Skotland@rr-research.no} ; Kim Ekroos³ ; ^&dagger ; ; ^{kekroos@hotmail.com} ; Simona Kavaliauskiene¹ ; ² ; ⁴ ; Jonas Bergan¹ ; ² ; Dimple Kauhanen³ ; Tuulia Lintonen³ ; Kirsten Sandvig¹ ; ² ; ⁴

关键词：MS ; mass spectrometry ; GSL ; glycosphingolipid ; Cer ; ceramide ; Gb3 ; globotriaosylceramide ; GlcCer ; glucosylceramide ; LacCer ; lactosylceramide ; MRM ; multiple reaction monitoring

刊名：Journal of Molecular Biology

出版年：2016

8.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

9.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

10.Risk factors for severe clinical events in male and female patients with Fabry disease treated with agalsidase beta enzyme replacement therapy: Data from the Fabry Registry

关键词：eGFR ; estimated glomerular filtration rate ; ERT ; enzyme replacement therapy ; ESRD ; end-stage renal disease ; GL-3 ; globotriaosylceramide ; IEC ; independent ethics committee ; IRB ; institutional review board ; LVH ; left ventricular hypertrophy ; UP/Cr ; urinary protein/creatinine ratio

刊名：Molecular Genetics and Metabolism

出版年：2016

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