维普期刊资源整合服务平台（V6.5）

64篇 您的检索式：作者名="Lutz AM"

选中清除导出查看参考文献查看引证文献引用追踪共4页首页上一页第1页下一页末页 /4 跳转

	题名	作者	年代	出处	被引量
1	Partial external biliary diversion in bile salt export pump deficiency: Association between outcome and mutation显示文摘AIM To investigate the relation of two different mutations to the outcome of partial external biliary diversion(PEBD)in severe bile salt export pump(BSEP) deficiency.METHODS Mutations in the gene encoding BSEP leading to severe BSEP deficiency in two unrelated patients were identified by genomic sequencing. Native liver biopsies and transiently transfected human embryonic kidney(HEK) 293 cells expressing either wild-type or mutated BSEP were subjected to immunofluorescence analysis to assess BSEP transporter localization. Bile acid profiles of patient and control bile samples were generated by ultra-performance liquid chromatographytandem mass spectrometry. Wild-type and mutant BSEP transport of [~3H]-labeled taurocholate(TC) and taurochenodeoxycholate(TCDC) was assessed by vesicular transport assays.RESULTS A girl(at 2 mo) presented with pruritus, jaundice and elevated serum bile salts(BS). PEBD stabilized liver function and prevented liver transplantation. She was heterozygous for the BSEP deletion p.T919 del and the nonsense mutation p.R1235 X. At the age of 17 years relative amounts of conjugated BS in her bile were normal, while total BS were less than 3% as compared to controls. An unrelated boy(age 1.5 years) presenting with severe pruritus and elevated serum BS was heterozygous for the same nonsense and another missense mutation, p.G1032 R. PEBD failed to alleviate pruritus, eventually necessitating liver transplantation. BS concentration in bile was about 5% of controls. BS were mainly unconjugated with an unusual low amount of chenodeoxycholate derivatives(< 5%). The patients' native liver biopsies showed canalicular BSEP expression. Both BSEP p.T919 del and p.G1032 R were localized in the plasma membrane in HEK293 cells. In vitro transport assays showed drastic reduction of transport by both mutations. Using purified recombinant BSEP as quantifiable reference, per-molecule transport rates for TC and TCDC were determined to be 3 and 2 BS molecules per wild-type BSEP transporter per minute, respectively.CONCLUSION In summary, our findings suggest that residual function of BSEP as well as substrate specificity influence the therapeutic effectiveness of PEBD in progressive familial intrahepatic cholestasis type 2(PFIC-2).	Philipp Ellinger Jan Stindt Carola Droge Katharina Sattler Claudia Stross Stefanie Kluge Diran Herebian Sander HJ Smits Martin Burdelski Sebastian Schulz-Jürgensen Antje Ballauff Jan Schulte am Esch Ertan Mayatepek Dieter Haussinger Ralf Kubitz Lutz Schmitt	2017	World Journal of Gastroenterology2017，23，29：	4
2	Mr imaging of theknee: position related changes of the menisci in asymptomaticvolunteers显示文摘	Boxheimer L Lutz AM Treiber K	2004	Invest Radiol2004，39，5：	1
3	MR imaging of the knee:position related changes of the menisci in asymptomatic volunteers显示文摘	Boxheimer L Lutz AM Treiber K	2006	Radiology2006，3，：	1
4	Mapping of hepatic vascular anatomy: dynamic contrast-enhanced parallel MR imaging compared with 64 detector row CT 显示文摘	Heilmaier C Sutter R Lutz AM	2007	Radiology2007，245，3：	1
5	Duabtargeted contrast agent for US assessment of tumor angiogenesis in vivo显示文摘	Willmann JK Lutz AM Paulmurugan R	2008	Radiology2008，248，：	1
6	MR imaging of the knee:position related changes of the menisci in asymptomatic volun-teers显示文摘	Boxheimer L Lutz AM Treiber K		0，，5：	1
7	Early predictors of selfbiting in socially-housed rhesus macaques (Macaca mulatta) 显示文摘	Lutz CK Davis EB Ruggiero AM	2007	Am J Primatol2007，69，5：	1
8	Assessment of the ab-dominal aorta and its visceral branches by contrast-enhanced dynamic volumetric hepatic parallel magnetic resonance ima-ging:feasibility,reliability and accuracy显示文摘	Werder R Nanz D Lutz AM	2007	Eur Radiol2007，17，：	1
9	MR imaging of theknee:position related changes of the menisci in asymptomaticvolunteers显示文摘	Boxheimer L Lutz AM Treiber K	2004	Invest Radiol2004，39，：	1
10	Detection of synovial macrophages in an experimental rabbit model of antigen-induced arthritis:ultrasmall superparamagnetic iron oxide-enhanced MR imaging显示文摘	Lutz AM Seemayer C Corot C	2004	Radiology2004，233，1：	1
11	Dual targeted contrast agent for US assessment of tumor angiogenesis in vivo显示文摘	Willmann JK Lutz AM Paulimurugran R		0，，03：	1
12	Earlier detection of breast cancer with ultrasound molecular imaging in a transgenic mouse model显示文摘	Bachawal SV Jensen KC Lutz AM		0，，6：	1
13	Earlier detection of breast cancer with ultrasound molecular imaging in a transgenic mouse model显示文摘	Bachawal SV Jensen KC Lutz AM	2013	Cancer Res2013，73，：	1
14	MR imaging of the knee:position related changes of the menisci in asymptomatic volunteers显示文摘	Boxheimer L Lutz AM Treiber K		0，，05：	1
15	Detection of synovial macrophages in an experimental rabbit model of antigen-induced arthritis: ultrasmall superparamagnetic iron oxide-enhanced MR imaging显示文摘	Lutz AM Seemayer C Corot C	2004	Radiology2004，233，1：	1
16	Systemic spread of meconium peritonitis显示文摘	Patton WL Lutz AM Willmann JK	1998	Pediatr Radiol1998，28，9：	1
17	Dual-targeted contrast a gent for US assessment of tume angiogenesis in vivo 显示文摘	Willmann JK Lutz AM Paulmuruga R	2008	Radiology2008，248，3：	1
18	Detection of synovial macrophages in an experimental rabbit model of antigen-induced arthritis:ultrasmall superparamagnetic iron oxide-enhanced MR imaging显示文摘	Lutz AM Seemayer C Corot C	2004	Radiology2004，233，1：	1
19	Mapping of hepatic vascular a- natomy: dynamic contrast-enhanced parallel MR imaging compared with 64 detector row CT显示文摘	Heilmaier C Sutter R Lutz AM	2007	Radiology2007，45，：	1
20	Hepatocellular Carcinoma in Cirrhosis:Enhancement Patterns at Dynamic Gadolinium-and Superparamagnetic Iron Oxide-enhanced T1-weighted MR Imaging显示文摘	Lutz AM Willmann JK Goepfert K	2005	Radiology2005，237，3：	1

返回顶部每页显示：

共4页首页上一页第1页下一页末页 /4 跳转

互联网出版许可证新出网证(渝)字10号全国400电话 - 免长途话费

用户提示