维普期刊资源整合服务平台（V6.5）

46篇 您的检索式：作者名="Burdelski"

选中清除导出查看参考文献查看引证文献引用追踪共3页首页上一页第1页下一页末页 /3 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	The wide spectrum of multidrug resistance 3 deficiency: From neonatal cholestasis to cirrhosis of adulthood显示文摘	Emmanuel Jacquemin J. Marleen L. De Vree Danièle Cresteil Etienne M. Sokal Ekkehard Sturm Micheline Dumont George L. Scheffer Marianne Paul Martin Burdelski Piter J. Bosma Olivier Bernard Michelle Hadchouel Ronald P.J. Oude Elferink	2001	Gastroenterology2001，，6：	2
3	An 18 kDa scaffold protein is critical for Staphylococcus epidermidis biofilm formation显示文摘	Decker R Burdelski C Zobiak M	2015	PLoS Pathog2015，11，10：	1
4	Selection of the living liver donor显示文摘	Sterneck MR Fischer L Nischwitz U Burdelski M Kjer S. Latta A Malago M Petersen J Pothmann W Rogiers X		0，，：	1
5	Crossing the Rubicon: When Pancreatic Resection with Curative Intent Ends in an R2 Status显示文摘	Maximilian Bockhorn MD Guellue Cataldegirmen MD Asad Kutup MD Andreas Marx MD Christoph Burdelski MD Jogesh K. Vashist MD Oliver Mann MD Lena Liebl MD Alexandra K?nig MD Jakob R. Izbicki MD Emre F. Yekebas MD	2009	Annals of Surgical Oncology2009，，5：	1
6	In Situ Splitting of Cadaveric Livers: The Ultimate Expansion of a Limited Donor Pool显示文摘	Xavier Rogiers Massimo Malagó Karim Gawad Karl W. Jauch Michael Olausson Wolfram T. Knoefel Matthias Gundlach Atef Bassas Lutz Fischer Martina Sterneck Martin Burdelski Christoph E. Broelsch	1996	Annals of Surgery1996，，3：	1
7	Induction of Staphylococcus epidermidis biofilm formation via proteolytic processing of the accumulation-associated protein by staphylococcal and host proteases 显示文摘	Rohde H Burdelski C Bartscht K	2005	Mol Microbiol2005，55，6：	1
8	Induction of Staph-ylococcus epidermidis biofilm formation via proteolytic processing of the accumulation-associated protein by staphylococ cal and host proteases显示文摘	Rohde H Burdelski C Bartscht K	2005	Mol Microbiol2005，55，：	1
9	Treatment of inherited metabolic disorders by liver transplantation 显示文摘	Rodeck B Latta A	1991	J Inherited Metab Dis1991，14，3：	1
10	Living related liver transplantation: 36 cases at the University of Hamberg 显示文摘	Rogiers X Burdelski M	1994	Transplant Proc1994，26，6：	1
11	Cytoplasmic ac- cumulation of sequestosome 1 (p62) is a predictor of biochemical recmTence, rapid tumor cell proliferation, and genomic instability in prostate cancer显示文摘	Burdelski C Reiswich V Hube - Magg C	2015	Clin Cancer Res2015，21，15：	1
12	One Hundred Thirty-Two Consecutive Pediatric Liver Transplants Without Hospital Mortality: Lessons Learned and Outlook for the Future显示文摘	Dieter C. Broering Jong-Sun Kim Teresa Mueller Lutz Fischer Rainer Ganschow Turan Bicak Lars Mueller Christian Hillert Christian Wilms Bernd Hinrichs Knut Helmke Werner Pothmann Martin Burdelski Xavier Rogiers	2004	Annals of Surgery2004，，6：	1
13	Induction of Staphylococcus epidermidis biofilm formation via proteolytic processing of the accumulation-associated protein by staphylococcal and host proteases 显示文摘	Rohde H Burdelski C Bartscht K	2005	Mol Microbiol2005，55，：	1
14	Induction of Staphylococcus epidermidis biofilm formation via proteolytic processing of the accumulation-associated protein by staphylococcal and host proteases显示文摘	ROHDEH BURDELSKI C BARTSCHTK		0，，06：	1
15	Living donor for liver transplantation显示文摘	Broelsch CE Burdelski M Rogiers X	1994	Hepatology1994，20，12：	1
16	Predictors of one-year pretransplant survival in patients with cirrhosis显示文摘	Oellerich M Burdelski M Lautz HU	1991	Hepatology1991，14，6：	1
17	Predictors of Oneyear Pretransplant Survival in Patients with Cirrhosis显示文摘	Oellerieh M Burdelski M Lautz H U	1991	Hepatology1991，14，：	1
18	Treatment of inherited metabolic disorders by liver transplantation显示文摘	Burdelski M Rodeck B Latta A	1991	J Inherited Metab Dis1991，14，：	1
19	Expression and localization of hepatobiliary transport proteins in progressive familial intrahepatic cholestasis显示文摘	Keitel V Burdelski M Warskulat U	2005	Hepatology2005，41，5：	1
20	Multivisceral resections in pancreatic cancer:identification of risk factors显示文摘	Burdelski CM Reeh M Bogoevski D		0，，12：	1

返回顶部每页显示：

共3页首页上一页第1页下一页末页 /3 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