搜索页_+Niemann-Pick+C+disease

设为首页

收藏本站

网站地图 | English | 公务邮箱

About the library

Background
History
Leadership
Organization

Readers' Guide

Opening Hours
Collections
Help Via Email

Publications

Electronic Information Resources

外文资源

Wiley电子期刊(4)	SpringerLink电子期刊(175)	NATURE电子期刊(8)	ACS电子期刊(9)
Springer电子图书(2)	ProQuest学位论文(1)	Elsevier电子期刊(567)

在“Elsevier电子期刊”中，命中：567条，耗时：0.0699671 秒

在所有数据库中总计命中：766条

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.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

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.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

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.Lysosomal acid lipase deficiency: Expanding differential diagnosis

作者：Vassili Valayannopoulos^a ; ^{vassilival.boston@gmail.com} ; Eugen Mengel^b ; Anais Brassier^a ; Gregory Grabowski^c

关键词：Lysosomal acid lipase deficiency ; Lysosomal storage disease ; Microvesicular steatosis ; Dyslipidemia ; Enzyme replacement therapy ; Hepatomegaly

刊名：Molecular Genetics and Metabolism

出版年：2017

8.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

9.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

10.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

按检索点细分(567)

题名(298)

关键词(181)

文摘(327)

按出版年细分(567)

2027年及以后(32)

2017年(17)

2016年(52)

2015年(16)

2013年(36)

2012年(37)

2011年(46)

2010年(38)

2009年(46)

2008年(21)

2007年(29)

2006年(19)

2005年(20)

2004年(23)

2003年(10)

2002年(16)

2001年(16)

2000年(28)

2000年及以前(65)