|
|
|
题名
|
作者
|
年代
|
出处
|
被引量
|
| 1 | Molecular overview of progressive familial intrahepatic cholestasis显示文摘Cholestasis is a clinical condition resulting from the imapairment of bile flow.This condition could be caused by defects of the hepatocytes,which are responsible for the complex process of bile formation and secretion,and/or caused by defects in the secretory machinery of cholangiocytes.Several mutations and pathways that lead to cholestasis have been described.Progressive familial intrahepatic cholestasis(PFIC)is a group of rare diseases caused by autosomal recessive mutations in the genes that encode proteins expressed mainly in the apical membrane of the hepatocytes.PFIC 1,also known as Byler’s disease,is caused by mutations of the ATP8B1 gene,which encodes the familial intrahepatic cholestasis 1 protein.PFIC 2 is characterized by the downregulation or absence of functional bile salt export pump(BSEP)expression via variations in the ABCB11 gene.Mutations of the ABCB4 gene result in lower expression of the multidrug resistance class 3 glycoprotein,leading to the third type of PFIC.Newer variations of this disease have been described.Loss of function of the tight junction protein 2 protein results in PFIC 4,while mutations of the NR1H4 gene,which encodes farnesoid X receptor,an important transcription factor for bile formation,cause PFIC 5.A recently described type of PFIC is associated with a mutation in the MYO5B gene,important for the trafficking of BSEP and hepatocyte membrane polarization.In this review,we provide a brief overview of the molecular mechanisms and clinical features associated with each type of PFIC based on peer reviewed journals published between 1993 and 2020. | Sriram Amirneni Nils Haep Mohammad A Gad Alejandro Soto-Gutierrez James E Squires Rodrigo MFlorentino | 2020 | World Journal of Gastroenterology2020，26，47： | 17 |
| 2 | Lateral performance of cold-formed steel-framed domestic structures 显示文摘 | Gad E F Duffield C F Hutchinson G L | 1999 | Engineering Structures1999，21，： | 1 |
| 3 | 显示文摘 | Hamed A M El-Sharkawy I I | 2001 | Rene wable Energy2001，22，4： | 1 |
| 4 | Water content, organic carbon and dry bulk density in flooded sediments显示文摘 | Avnimelech Y Gad R Leon E M | 2001 | Aquacultural engineering2001，25，： | 1 |
| 5 | The use of wood pulp and radiation modified starch in wastewater treatment显示文摘 | Abdel Aal S E Gad Y Dessouki A M | 2006 | J Appl Polym Sci2006，99，5： | 1 |
| 6 | Water content,organic carbon and dry bulk density in flooded sediments 显示文摘 | Avnimelech Y Gad R Leon E M | 2001 | Aquacultural engineering2001，25，1： | 1 |
| 7 | How does duration and dispersion of atrial refractoriness influence persistence of atrial fibrillation显示文摘 | Leistad E Subudhayangkul S Ungab GAD | 1999 | Eur Heart J1999，20，1： | 1 |
| 8 | Modal analysis of steelframed residential structures for application to seismic design 显示文摘 | Gad E F Chandler A M | 2001 | Journal of Vibration and Control2001，17，1： | 1 |
| 9 | On the radial vibra tion of ball bearings (computer simulation)显示文摘 | Fukata S Gad E H Kondou T | 1985 | Bulletin of the JSME1985，28，239： | 1 |
| 10 | Simulation and sensitivity analysis of nonu niform transmission lines using integrated congruence trans- form显示文摘 | Gad E Nakhla M | 2005 | IEEE Transactions on Advanced Packaging2005，28，9： | 1 |
| 11 | Lateral Behavior of Plasterboard-clad Residential Steel Frames显示文摘 | Gad E F Chandeler A M Duffield C F | 1999 | Journal of Structural Engineering1999，125，1： | 1 |
| 12 | Use of rice straw and radiation-modified maize starch/acrylonitrile in the treatment of wastewater 显示文摘 | Abdel-Aal S E Gad Y H Dessouk i A M | 2006 | Journal of Hazardous Materials2006，，13： | 1 |
| 13 | Polysaccharide krestin is a novel TLR2 agonist that mediates inhibition of tumor growth via stimula- tion of CD8 T cells and NK cells 显示文摘 | Lu H Yang Y Gad E | 2011 | Clin Cancer Res2011，17，1： | 1 |
| 14 | Characterization of T reg-ulatory cells,induced by immature dendrtic cells which inhibit en-teroantigen reactive colitis-inducing T-cell responses in vitro and invivo显示文摘 | Gad M Kristensen N N Kury E | 2004 | Immunol2004，113，4： | 1 |
| 15 | Inversion of DC resistivity data usingneural network显示文摘 | Gad E Q Keisuke U | 2001 | Geophysical Prospecting2001，49，4： | 1 |
| 16 | Development of Test Method for Determining Plasterboard Bracing Performance 显示文摘 | LIEW Y L GAD E F DUFFIELD C F | 2004 | Journal of Structural Engineering2004，130，7： | 1 |
| 17 | On the radial vibration of ballbearings(computer simulation)显示文摘 | Fukata S Gad E H Tamura H | 1985 | Bulletin of the JSME1985，28，： | 1 |
| 18 | Application of a solar desiccant/collector system for water recovery from atmospheric air显示文摘 | H E Gad AM Hamed | 2001 | Renewable Energy2001，，22： | 1 |
| 19 | On the radial vibration of ball bearings( computer simulation ) 显示文摘 | Fukata S Gad E H Kondou T | 1985 | Bulletin of the JSME1985，，28： | 1 |
| 20 | On the radial vibration of ball bearings (computer simulation)显示文摘 | Fukata S Gad E H Kondou T | 1985 | Bulletin of the JSME 281985，，： | 1 |
您所在的机构暂未开通此部分的功能，请联系管理员