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在“Elsevier电子期刊”中，命中：11条，耗时：小于0.01 秒

在所有数据库中总计命中：110条

1.Gaucher disease: Progress and ongoing challenges

作者：Pramod K. Mistry^a ; ^{Pramod.mistry@yale.edu} ; Grisel Lopez^b ; ^{glopez@mail.nih.gov} ; Raphael Schiffmann^c ; ^{Raphael.Schiffmann@BSWHealth.org} ; Norman W. Barton^d ; ^{nbarton@shire.com} ; Neal J. Weinreb^e ; ^{boneal@winning.com} ; Ellen Sidransky^b ; ^{sidranse@mail.nih.gov}

关键词：ERT ; enzyme replacement therapy ; SRT ; substrate reduction therapy ; iPSC ; induced pluripotent stem cells ; GBA1 ; the glucocerebrosidase gene ; CBE ; conduritol β-epoxide ; HSP ; heat shock protein ; ER ; endoplasmic reticulum ; HDAC ; histone deacetylase ; NIH ; National Institutes of Health ; FDA ; Food and Drug Administration

刊名：Molecular Genetics and Metabolism

出版年：2017

2.Leukocyte glucocerebrosidase and β-hexosaminidase activity in sporadic and genetic Parkinson disease

作者：Han-Joon Kim^a ; Beomseok Jeon^a ; ^{brain@snu.ac.kr" class="auth_mail" title="E-mail the corresponding author} ; Junghan Song^b ; ^c ; Woong-Woo Lee^d ; Hyeyoung Park^a ; Chae-Won Shin^a

关键词：Parkinson disease ; Leukocyte ; β-Glucocerebrosidase ; β-hexosaminidase ; Gaucher disease

刊名：Parkinsonism & Related Disorders

出版年：2016

3.Progranulin Recruits HSP70 to β-Glucocerebrosidase and Is Therapeutic Against Gaucher Disease

作者：Jinlong Jian^a ; Qing-Yun Tian^a ; Aubryanna Hettinghouse^a ; Shuai Zhao^a ; Helen Liu^a ; Jianlu Wei^a ; Gabriele Grunig^b ; Wujuan Zhang^c ; Kenneth D.R. Setchell^c ; Ying Sun^d ; Herman S. Overkleeft^e ; Gerald L. Chan^f ; Chuan-ju Liu^a ; ^g ; ^{chuanju.liu@nyumc.org}

关键词：Progranulin ; HSP70 ; β-glucocerebrosidase ; Gaucher disease ; Lysosomal storage diseases ; Pcgin

刊名：EBioMedicine

出版年：2016

4.A peptide-linked recombinant glucocerebrosidase for targeted neuronal delivery: Design, production, and assessment

作者：Paul A. Gramlich^a ; ^b ; ^{pgramli1@jhu.edu" class="auth_mail" title="E-mail the corresponding author} ; ^{paul.gramlich@gmail.com" class="auth_mail" title="E-mail the corresponding author} ; Wendy Westbroek^c ; Ricardo A. Feldman^d ; Ola Awad^d ; Nicholas Mello^b ; ^e ; Mary P. Remington^b ; Ying Sun^f ; ^g ; Wujuan Zhang^h ; Ellen Sidransky^c ; Michael J. Betenbaugh^a ; Paul S. Fishman^b ; ⁱ

关键词：GCase ; glucocerebrosidase ; GD ; Gaucher&rsquo ; s disease ; RDP ; rabies-derived peptide ; TTC ; tetanus toxin fragment C ; IgAh ; peptide from flexible hinge region of IgA ; ERT ; enzyme replacement therapy ; GlcCer ; glucosylceramide ; GlcSph ; glucosylsphingosine ; 4-MUG ; 4-Methylumbelliferyl β-d-glucopyranoside ; CBE ; conduritol B epoxide ; GCKO cell line ; glucocerebrosidase-knockout neuronal cell line

刊名：Journal of Biotechnology

出版年：2016

5.Glycosphingolipid analysis in a naturally occurring ovine model of acute neuronopathic Gaucher disease

作者：Litsa Karageorgos^a ; ^{litsa.karageorgos@sahmri.com" class="auth_mail" title="E-mail the corresponding author} ; Leanne Hein^a ; Tina Rozaklis^a ; Melissa Adams^a ; Stephen Duplock^a ; Marten Snel^a ; Kim Hemsley^a ; Tim Kuchel^b ; Nicholas Smith^c ; ^d ; John J. Hopwood^a

关键词：BMP ; bis(monoacylglycero)phosphate ; Cer ; ceramide ; DHC ; dihexosylceramide ; GalCer ; galactosylceramide ; GCase ; β-glucocerebrosidase ; GBA1 ; β-glucocerebrosidase gene ; GlcCer ; glucosylceramide ; GlcSph ; glucosylsphingosine ; PC ; phosphatidylcholine ; PE ; phosphatidylethanolamine ; PG ; phosphatidylglycerol ; PI ; phosphatidylinositol ; SM ; sphingomyelin ; SEM ; standard error of the mean ; THC ; trihexosylceramide

刊名：Neurobiology of Disease

出版年：2016

6.α-Synuclein dimerization in erythrocytes of Gaucher disease patients: correlation with lipid abnormalities and oxidative stress

作者：Marina Moraitou^a ; Georgia Dermentzaki^b ; Evangelia Dimitriou^a ; Ioannis Monopolis^a ; Nick Dekker^c ; Hans Aerts^c ; Leonidas Stefanis^b ; ^d ; Helen Michelakakis^a ; ^{inchildh@otenet.gr" class="auth_mail" title="E-mail the corresponding author}

关键词：α-Syn ; α-Synuclein ; C16 ; 0 DMA/C160 ; palmitate dimethylacetal/palmitate ; Cer ; ceramide ; GBA ; glucocerebrosidase gene ; GD ; Gaucher disease ; Glcase ; β-glucocerebrosidase ; GlcCer ; glucosylceramide ; iPS ; induced pluripotent stem (cells) ; MDA ; malonyldialdehyde ; PD ; Parkinson&rsquo ; s disease ; RB ; Cred blood cell

刊名：Neuroscience Letters

出版年：2016

7.Triplex tandem mass spectrometry assays for the screening of 3 lysosomal storage disorders in a Korean population

作者：Sung Eun Cho^a ; Joung Ryull Kwak^a ; Hyojung Lee^a ; Dong Hee Seo^a ; Junghan Song^b ; ^c ; ^{songjhcp@snu.ac.kr" class="auth_mail" title="E-mail the corresponding author}

关键词：GAA ; acid α-glucosidase (Pompe disease) ; GLA ; acid α-galactosidase (Fabry disease) ; GBA ; acid β-glucocerebrosidase (Gaucher disease) ; CDC ; Centers for Disease Control and Prevention ; MRM ; multiple reaction monitoring ; S ; substrate

刊名：Clinica Chimica Acta

出版年：2016

8.From rare to common and back again: 60 years of lysosomal dysfunction

作者：Maria Francisca Coutinho ; ^{francisca_coutinho@yahoo.com" class="auth_mail" title="E-mail the corresponding author} ; ^{francisca.coutinho@insa.min-saude.pt" class="auth_mail" title="E-mail the corresponding author} ; Sandra Alves

关键词：SMase ; acid sphingomyelinase ; AD ; Alzheimer disease ; BBB ; blood&ndash ; brain barrier ; CD-MPR ; cation-dependent mannose 6-phosphate receptor ; CI-MPR ; cation-independent mannose 6-phosphate receptor ; CNS ; central nervous system ; CMA ; chaperone-mediated autophagy ; DLB ; dementia with Lewy bodies ; EE ; early endosome ; ER ; endoplasmic reticulum ; ERT ; enzyme replacement therapy ; FTLD ; frontotemporal lobar degeneration ; GD ; Gaucher disease ; GCase ; β-Glucocerebrosidase ; HSC70 ; heat shock cognate 70 ; LE ; la

刊名：Molecular Genetics and Metabolism

出版年：2016

9.Association Between Progranulin and Gaucher Disease

作者：Jinlong Jian^a ; Shuai Zhao^a ; Qing-Yun Tian^a ; Helen Liu^a ; Yunpeng Zhao^a ; Wen-Chi Chen^b ; Gabriele Grunig^b ; Paola A. Torres^c ; Betty C. Wang^c ; Bai Zeng^c ; Gregory Pastores^c ; Wei Tang^d ; Ying Sun^e ; Gregory A. Grabowski^e ; Max Xiangtian Kong^f ; Guilin Wang^g ; Ying Chen^h ; Fengxia Liangⁱ ; Herman S. Overkleeft^j ; Rachel Saunders-Pullman^k ; Gerald L. Chan^l ; Chuan-ju Liu^a ; ^m ; ^{chuanju.liu@nyumc.org" class="auth_mail" title="E-mail the corresponding author}

关键词：Progranulin ; β-Glucocerebrosidase ; Gaucher disease ; Lysosomal storage diseases

刊名：EBioMedicine

出版年：2016

10.ER stress and Parkinson's disease: Pathological inputs that converge into the secretory pathway

作者：Gabriela Mercado^a ; ^b ; ^{mariamercado@med.uchile.cl" class="auth_mail" title="E-mail the corresponding author} ; ^{gabriela.mercado.guerra@gmail.com" class="auth_mail" title="E-mail the corresponding author} ; Valentina Castillo^a ; ^b ; Paulina Soto^a ; ^b ; Anita Sidhu^c

关键词：PD ; Parkinson's disease ; SNpc ; substantia nigra pars compacta ; LB ; Lewy bodies ; αSyn ; αSynuclein ; ER ; endoplasmic reticulum ; ERAD ; ER-Associated Degradation ; PINK1 ; PTEN-induced putative kinase 1 ; ATP13A2 ; lysosomal P-type transport ATPase ; LRRK2 ; leucine-rich repeat kinase 2 ; VPS35 ; vacuolar protein sorting-35 protein ; UCHL-1 ; ubiquitin carboxy-terminal hydrolase L1 ; GCase ; β-glucocerebrosidase ; DAT ; dopamine transporter ; SNARE ; soluble NSF attachment protein receptor ; TFEB ; transcription facto

刊名：Brain Research

出版年：2016

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