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在“Elsevier电子期刊”中，命中：1,580条，耗时：小于0.01 秒

1.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

2.Types A and B Niemann-Pick disease

作者：Edward H. Schuchman ; ^{edward.schuchman@mssm.edu} ; Robert J. Desnick

关键词：Niemann-Pick ; Acid sphingomyelinase ; Sphingomyelin ; Mouse model ; Enzyme Replacement Therapy

刊名：Molecular Genetics and Metabolism

出版年：2017

3.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

4.The new obesity-associated protein, neuronal growth regulator 1 (NEGR1), is implicated in Niemann-Pick disease Type C (NPC2)-mediated cholesterol trafficking

作者：Hyejin Kim^a ; Younghwa Chun^a ; Lihua Che^a ; Jeongbeom Kim^a ; Sungjoong Lee^b ; Soojin Lee^a ; ^{leesoojin@cnu.ac.kr}

关键词：Neuronal growth regulator 1 ; Niemann-Pick disease ; Type C2 ; Lipid raft ; Cholesterol ; Obesity

刊名：Biochemical and Biophysical Research Communications

出版年：2017

5.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

6.A saposin deficiency model in Drosophila: Lysosomal storage, progressive neurodegeneration and sensory physiological decline

作者：Samantha J. Hindle^a ; ¹ ; Sarita Hebbar^b ; ² ; Dominik Schwudke^b ; ³ ; Christopher J.H. Elliott^a ; Sean T. Sweeney^a ; ^{sean.sweeney@york.ac.uk}

关键词：PSAP ; prosaposin ; ERG ; electroretinograms ; LSD ; lysosomal storage disease ; NPC ; Niemann-Pick type C ; GFP ; green fluorescent protein ; RT-PCR ; reverse transcription PCR ; TEM ; transmission electron microscopy ; MVB ; multivesicular body ; MLB ; multilamellar body ; HSAN1 ; hereditary sensory and autonomic neuropathy type 1

刊名：Neurobiology of Disease

出版年：2017

7.Decreasing SMPD1 activity in BEAS-2B bronchial airway epithelial cells results in increased NRF2 activity, cytokine synthesis and neutrophil recruitment

作者：Elyse MacFadden-Murphy ; Lucie Roussel ; Guy Martel ; Julie Bé ; rubé ; Simon Rousseau ; ^{simon.rousseau@mcgill.ca}

关键词：Niemann-Pick disease ; Acidic sphingomyelinase ; Airway epithelium ; Interleukins ; Pseudomonas aeruginosa ; Reactive oxygen species

刊名：Biochemical and Biophysical Research Communications

出版年：2017

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.Identification of relevant clinical niches in rare inherited metabolic diseases: Niemann-Pick disease type C as a model

作者：Chris J. Hendriksz^a ; ^b ; Stefan A. Kolb^c

刊名：Molecular Genetics and Metabolism

出版年：2017

10.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

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