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| 1 | Current and future therapies for inherited cholestatic liver diseases显示文摘Familial intrahepatic cholestasis(FIC) comprises a group of rare cholestatic liver diseases associated with canalicular transport defects resulting predominantly from mutations in ATP8B1, ABCB11 and ABCB4. Phe-notypes range from benign recurrent intrahepatic cholestasis(BRIC), associated with recurrent cholestatic attacks, to progressive FIC(PFIC). Patients often suffer from severe pruritus and eventually progressive cholestasis results in liver failure. Currently, first-line treatment includes ursodeoxycholic acid in patients with ABCB4 deficiency(PFIC3) and partial biliary diversion in patients with ATP8B1 or ABCB11 deficiency(PFIC1 and PFIC2). When treatment fails, liver transplantation is needed which is associated with complications like rejection, post-transplant hepatic steatosis and recurrence of disease. Therefore, the need for more and better therapies for this group of chronic diseases remains. Here, we discuss new symptomatic treatment options like total biliary diversion, pharmacological diversion of bile acids and hepatocyte transplantation. Furthermore, we focus on emerging mutation-targeted therapeutic strategies, providing an outlook for future personalized treatment for inherited cholestatic liver diseases. | Wendy L van der Woerd Roderick HJ Houwen Stan FJ van de Graaf | 2017 | World Journal of Gastroenterology2017，23，5： | 11 |
| 2 | The Dynamic Pollen Tube Cytoskeleton: Live Cell Studies Using Actin-Binding and Microtubule- Binding Reporter Proteins显示文摘花粉试管在雌蕊以内伸长为授精把精子房间搬运到胚胎囊。生长专门在试管顶发生,显示花粉试管延伸一个很戏剧的极的房间生长过程。一个特点花粉试管特征它的细胞骨架,它精心地包括被组织并且动态肌动朊微细丝和微导管。花粉试管生长依赖于肌动朊细胞骨架;它的组织和规定被各种各样的途径广泛地检验了,包括荧光灯在实时房间标记肌动朊绑定蛋白质的蛋白质学习。用以前描述的 GFP-NtADF1 和 GFP-LlADF1,并且新肌动朊记者蛋白质 NtPLIM2b-GFP,我们重申占优势的肌动朊在伸长组织烟草和百合花粉试管,这长,沿着花粉试管胫的流的肌动朊电报,和包括更短的肌动朊电报的接近顶点的结构。肌动朊微细丝的接近顶点的收集经历动态变化,产生从 basket-shaped,的结构的外观或塑造漏斗,对一枚微妙的戒指像网孔。NtPLIM2b-GFP 为 Rho GTPases 与一个 guanine 核苷酸交换因素在联合被使用, AtROP-GEF1,说明这些肌动朊记者蛋白质的使用探索在极的细胞生长过程和它的肌动朊细胞骨架之间的连接。与肌动朊细胞骨架相反,微导管看起来不在在被子植物花粉试管支持极的房间生长过程起一个直接作用。从 Arabidopsis AtEB1 基于微导管结束绑定蛋白质使用微导管记者蛋白质, GFP-AtEB1,我们证明广泛的微导管在伸长联网改变动力学的度的花粉试管显示器。这些记者蛋白质提供万用的工具探索在极的花粉试管生长过程的主要结构、发信号的部件之间的功能的连接。 | Alice Y. Cheung Qiao-hong Duan Silvia Santos Costa Barend H.J. de Graaf Veronica S. DiStilio Jose Feijo Hen-Ming Wu | 2008 | Molecular Plant2008，1，4： | 10 |
| 3 | Anaerobic ammonium oxidation discovered in a denitrifying fluidized bed reactor显示文摘 | A. Mulder A.A. van de Graaf L.A. Robertson J.G. Kuenen | 1995 | FEMS Microbiology Ecology1995，，3： | 3 |
| 4 | Anaerobic ammonium oxidation discovered in a denitrifying fluidized bed reactor显示文摘 | A. Mulder A.A. van de Graaf L.A. Robertson J.G. Kuenen | 1995 | FEMS Microbiology Ecology1995，，3： | 3 |
| 5 | Physiological and Biochemical Basis of Clinical Liver Function Tests: A Review显示文摘 | Lisette T. Hoekstra Wilmar de Graaf Geert A. A. Nibourg Michal Heger Roelof J. Bennink Bruno Stieger Thomas M. van Gulik | 2013 | Annals of Surgery2013，，1： | 3 |
| 6 | Randomised comparison of morbidity after D1 and D2 dissection for gastric cancer in 996 Dutch patients显示文摘 | J.J. Bonenkamp I. Songun K. Welvaart C.J.H. van de Velde J. Hermans M. Sasako J.T.M. Plukker P. van Elk H. Obertop D.J. Gouma C.W. Taat J. van Lanschot S. Meyer P.W. de Graaf M.F. von Meyenfeldt H. Tilanus | 1995 | The Lancet . 1995 (8952)1995，，： | 2 |
| 7 | Autotrophlic growth of anaerobic ammonium-oxidizing microorganisms in a fluidized bed reactor 显示文摘 | Van de Graaf A A De Bruijn P Robertson L A | 1996 | Microbiology1996，142，8： | 2 |
| 8 | Systematic review of endoscopic mucosal resection versus transanal endoscopic microsurgery for large rectal adenomas显示文摘 | R. Barendse F. van den Broek E. Dekker W. Bemelman E. de Graaf P. Fockens J. Reitsma | 2011 | Endoscopy2011，，11： | 2 |
| 9 | Vertebroplasty versus conservative treatment in acute osteoporotic vertebral compression fractures (Vertos II): an open-label randomised trial显示文摘 | Caroline AH Klazen Paul NM Lohle Jolanda de Vries Frits H Jansen Alexander V Tielbeek Marion C Blonk Alexander Venmans Willem Jan J van Rooij Marinus C Schoemaker Job R Juttmann Tjoen H Lo Harald JJ Verhaar Yolanda van der Graaf Kaspar J van Everdingen Al | 2010 | 2010 (9746)2010，，9746： | 2 |
| 10 | Incisional inferior Lumbar hernia显示文摘 | De Graaf R Van Heesewijk JP | 2005 | JBR-BTR2005，88，3： | 1 |
| 11 | Speculation on whether a vaccine against cryptosporidiosis is a reality or fantasy 显示文摘 | De Graaf D C Spano F Petry F | 1999 | Int J Parasitol1999，29，8： | 1 |
| 12 | A comparison of 13C NMR measurements of the rates of glutamine synthesis and the tricarboxylic acid cycle during oral and intravenous administration of glucose显示文摘 | Mason G F Falk Petersen K de Graaf R A | 2003 | Brain Res Protoc2003，10，3： | 1 |
| 13 | Nuclear imaging techniques for the assessment of hepatic function in liver surgery and transplantation显示文摘 | de Graaf W Bennink R J Vetelainen R | 2010 | J Nucl Med2010，51，5： | 1 |
| 14 | Assessment of future remnant liver function using hepatobiliary scintigraphy in patients undergoing major liver resection显示文摘 | de Graaf W van Lienden KP Dinant S | 2010 | J Gastrointest Surg2010，14，2： | 1 |
| 15 | Anaerobic ammonium oxidation discovered in a denitrifying fluidized bed reactor显示文摘 | Mulder A Van de Graaf A A Robertson L A | 1995 | FEMS Microbiology Ecology1995，16，： | 1 |
| 16 | Quantitative determination of metabolic fluxs during coutilization of two carbon sources:comparative analysis with Corynebacterium glutamicum durin growth on acetate and glucose显示文摘 | Volker F Wendisch Albert A DE Graaf Hermann Sahm | 2000 | Journal of Biotechnology2000，，： | 1 |
| 17 | Functional expression of the epithelial Ca2+ channels (TRPV5 and TRPV6) requires association of the S100A10-annexin 2 complex 显示文摘 | van de Graaf SF Hoenderop JG Gkika D | 2003 | EMBO J2003，22，7： | 1 |
| 18 | In vivo quantification of parallel and bidirectional fiuxes in the anaplerosis of Corynebacterium glutamicum 显示文摘 | Petersen S de Graaf A A Eggeling L | 2000 | J Biol Chem2000，275，35： | 1 |
| 19 | A mutation in the fibroblast growth factor 14 gene is associated with autosomal dominant cerebellar ataxia显示文摘 | Van Swieten JC Brusse E de Graaf BM | 2003 | Am J Hum Genet2003，72，1： | 1 |
| 20 | Adverseeffects of propranolol when used in the treatment of hemangio -mas: a case series of 28 infants显示文摘 | de GRAAF M BREUR JM RAPHAEL MF | 2011 | J Am Acad Dermatol2011，65，2： | 1 |
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