搜索页_+Niemann-Pick+C1

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在“Elsevier电子期刊”中，命中：270条，耗时：0.0139933 秒

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1.Modeling Niemann Pick type C1 using human embryonic and induced pluripotent stem cells

作者：M. Paulina Ordoñ ; ez^a ; ^b ; ^{pordonez@ucsd.edu} ; John W. Steele^a

关键词：Niemann Pick type C1 ; Human embryonic stem cells ; Human induced pluripotent stem cells ; Neurodegeneration ; Models of disease ; Drug screening ; Autophagy

刊名：Brain Research

出版年：2017

2.Amelioration of non-alcoholic fatty liver disease with NPC1L1-targeted IgY or n-3 polyunsaturated fatty acids in mice

作者：Jin-Sik Bae^a ; ¹ ; Jong-Min Park^b ; ¹ ; Junghoon Lee^a ; Byung-Chul Oh^a ; Sang-Ho Jang^c ; Yun Bin Lee^b ; ^d ; Young-Min Han^b ; Chan-Young Ock^b ; Ji-Young Cha^a ; ^e ; ^{jycha1014@gachon.ac.kr} ; ^{jycha1014@gmail.com} ; Ki-Baik Hahm^b ; ^d ; ^{hahmkb@cha.ac.kr}

关键词：ABCG5/G8 ; ATP-binding cassette ; sub-family G5/G8 ; CYP7A1 ; cholesterol 7α-hydroxylase ; EZM ; ezetimibe ; HFD ; high fat diet ; NAFLD ; non-alcoholic fatty liver disease ; NPC1L1 ; Niemann Pick C1-like 1 ; PUFAs ; polyunsaturated fatty acids ; LXR ; liver X receptor

刊名：Metabolism

出版年：2017

3.Niemann-Pick disease, type C and Roscoe Brady

作者：Marc C. Patterson^a ; ^b ; ^{patterson.marc@mayo.edu} ; Steven U. Walkley^c ; ^{Steve.walkley@einstein.yu.edu}

关键词：Roscoe Brady ; Niemann-Pick disease ; Type C ; Cholesterol ; Oxysterols ; Gangliosides ; Biomarkers ; Filipin ; Cyclodextrin ; Miglustat

刊名：Molecular Genetics and Metabolism

出版年：2017

4.Defective macrophage phagocytosis and particle clearance in Niemann-Pick disease type C1 mice

作者：Stephanie K Newman^a ; Oscar CW Chen^b ; Lauren Morris^c ; David A Smith^d ; Nick Platt^e ; Frances M Platt^e

刊名：Molecular Genetics and Metabolism

出版年：2017

5.Intravenous and intrathecal treatment with HP-β-CD reduced disease biomarkers in a patient with Niemann-Pick disease type C1

作者：Nina Movsesyan^a ; Vanessa L. Wall^a ; Daniel S. Ory^b ; Raymond Wang^a

刊名：Molecular Genetics and Metabolism

出版年：2017

6.Niemann-Pick disease type C1(NPC1) is involved in resistance against imatinib in the imatinib-resistant Ph+ acute lymphoblastic leukemia cell line SUP-B15/RI

作者：Duolan Naren^a ; Jiahui Wu^a ; Yuping Gong^a ; ^{gongyuping2010@aliyun.com" class="auth_mail" title="E-mail the corresponding author} ; Tianyou Yan^a ; Ke Wang^b ; ^c ; Wenming Xu^b ; ^c ; Xi Yang^a ; Fangfang Shi^a ; Rui Shi^a

关键词：Niemann-Pick disease type C1 ; Drug resistance ; Drug transport ; Lysosome ; Leukemia ; Cholesterol regulation

刊名：Leukemia Research

出版年：2016

7.Extensive macrophage accumulation in young and old Niemann-Pick C1 model mice involves the alternative, M2, activation pathway and inhibition of macrophage apoptosis

作者：Gail Deutsch^a ; Akshay Muralidhar^b ; Ellen Le^b ; Ivan A. Borbon^b ; Robert P. Erickson^b ; ^c ; ^{erickson@peds.arizona.edu" class="auth_mail" title="E-mail the corresponding author}

关键词：BAL ; bronchoalveolar lavage ; ELISA ; enzyme-liked immunosorbent assay ; GFAP ; glial fibrillary acid protein ; GM-CSF ; granulocyte macrophage colony-stimulating factor ; H & E ; hematoxylin and eosin ; HPBCD ; hydroxypropyl-beta-cyclodextrin ; NPC1 ; Niemann&ndash ; Pick C 1 ; NPC2 ; Niemann&ndash ; Pick C 2 ; NSE ; neuron-specific enolase ; PAS ; periodic acid Schiff's ; PBS ; phosphate-buffered saline ; S1P ; Sphingosine-1-phosphate ; SEM ; standard error of the mean ; TUNEL ; terminal deoxynucleotidyl transferase-mediat

刊名：Gene

出版年：2016

8.Transgenic overexpression of Niemann-Pick C2 protein promotes cholesterol gallstone formation in mice

作者：Mariana Acuñ ; a¹ ; ⁵ ; ^&dagger ; ; Lila Gonzá ; lez-Hó ; dar¹ ; ^&dagger ; ; Ludwig Amigo¹ ; Juan Castro¹ ; M. Gabriela Morales¹ ; Gonzalo I. Cancino² ; Albert K. Groen³ ; Juan Young⁴ ; Juan Francisco Miquel¹ ; ⁵ ; Silvana Zanlungo¹ ; ⁵ ; ^{silvana@med.puc.cl" class="auth_mail" title="E-mail the corresponding author}

关键词：NPC2 ; Niemann-Pick C2 ; NPC1 ; Niemann-Pick C1 ; NPC2(h/h) ; Niemann-Pick C2-deficient hypomorph mice ; ABCG5/G8 ; adenosine triphosphate&ndash ; binding cassettes G5 and G8 ; ConA ; Concanavalin A ; Npc2.Tg ; Niemann-Pick C2 transgenic mice ; PCR ; polymerase chain reaction ; qPCR ; quantitative polymerase chain reaction ; HEK ; human embryonic kidney ; BSA ; bovine serum albumin ; ALT ; alanine transaminase ; Abcb11 ; ATP-binding cassette ; sub-family B member 11 ; Abcb4 ; ATP-binding cassette ; sub-family B member 4 ; CS

刊名：Journal of Hepatology

出版年：2016

9.Stimulation of adenosine A_2A receptors reduces intracellular cholesterol accumulation and rescues mitochondrial abnormalities in human neural cell models of Niemann-Pick C1

作者：A. Ferrante^a ; ^{antonella.ferrante@iss.it" class="auth_mail" title="E-mail the corresponding author} ; C. De Nuccio^b ; R. Pepponi^a ; S. Visentin^b ; A. Martire^a ; A. Bernardo^b ; L. Minghetti^b ; P. Popoli^a

关键词：Niemann-Pick C ; Adenosine A2A receptor ; Cholesterol ; Mitochondrial membrane potential ; Neuroblastoma cell line ; Oligodendroglial cell line

刊名：Neuropharmacology

出版年：2016

10.Mitochondrial dysfunction in fibroblasts derived from patients with Niemann-Pick type C disease

作者：Marcin Woś^a ; Joanna Szczepanowska^a ; Sławomir Pikuła^a ; Anna Tylki-Szymańska^b ; Krzysztof Zabłocki^a ; Joanna Bandorowicz-Pikuła^a ; ^{j.bandorowicz-pikula@nencki.gov.pl" class="auth_mail" title="E-mail the corresponding author}

关键词：Cholesterol ; Accumulation ; Mitochondrial dysfunction ; Organization of mitochondrial network ; Human Niemann-Pick type C1 mutant fibroblasts

刊名：Archives of Biochemistry and Biophysics

出版年：2016

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