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75篇 您的检索式：作者名="Ballauff"

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	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	Aktualisierte Leitlinie zur Diagnostik und Therapie der Colitis ulcerosa 2011 – Ergebnisse einer Evidenzbasierten Konsensuskonferenz显示文摘	A. Dignass J. Prei? D. Aust F. Autschbach A. Ballauff G. Barretton B. Bokemeyer S. Fichtner-Feigl S. Hagel K. Herrlinger G. Jantschek A. Kroesen W. Kruis T. Kucharzik J. Langhorst M. Reinshagen G. Rogler D. Schleiermacher C. Schmidt S. Schreiber H. Schulz	2011	Z Gastroenterol2011，，09：	2
3	Spherical polyelectrolyte brushes显示文摘	BALLAUFF M	2007	ProgPolym Sci>2007，32，10：	1
4	Synthesis of spherical polyelectrolyte brushes by photoemulsion polymerization显示文摘	Guo X Weiss A Ballauff M	1999	Macromolecules1999，32，19：	1
5	Secondary structure analysis of proteins embedded in spherical polyelectrolyte brushes by FT-IR spectroscopy 显示文摘	Wittemann A Ballauff M	2004	Anal Chem2004，76，：	1
6	Adsorptionof proteins on spherical polyelectrolyte brushes in aqueous so-lution显示文摘	WITTEMANN A HAUPT B BALLAUFF M	2003	Phys Chem Chem Phys2003，5，：	1
7	Spatial correlation of spherical polyelectrolyte brushes in salt-free solution as observed by small-angle x-ray scattering显示文摘	Robillard I) Guo X Ballauff M	2000	Macromolecules2000，33，24：	1
8	NOD2 mutations predict the risk for surgery in pediatric-onset Crohn’s disease显示文摘	Martin Lacher Johanna Helmbrecht Sebastian Schroepf Sibylle Koletzko Antje Ballauff Martin Classen Holm Uhlig Jochen Hubertus Dominik Hartl Peter Lohse Dietrich von Schweinitz Roland Kappler	2010	Journal of Pediatric Surgery2010，，8：	1
9	Spatial dimensions of colloidalpolyelectrolyte brushes as determined by dynamic light scat-tering显示文摘	GUO X BALLAUFF M	2000	Langmuir2000，16，23：	1
10	Polyelec-trolyte brushes显示文摘	RUHE J BALLAUFF M BIESALSKI M	2004	Adv Polym Sci2004，165，：	1
11	Heterotopic auxiliary liver transplantation in a 3-year-old boy with acute liver failure and aplastic anemia 显示文摘	Roll C Ballauff A Lange R	1997	Transplantation1997，64，4：	1
12	Thermosensitive core-shell microgels: From colloidal model systems to nanoreaetors显示文摘	LU Y BALLAUFF M	2011	Progress in Polymer Science2011，36，6：	1
13	Heterotopic auxiliary liver transplantation in a 3-year-old boy with acute liver failure and aplastic anemia显示文摘	ROLL C BALLAUFF A LANGE R	1997	Transplantation1997，64，4：	1
14	Single nanocrystals of platinum prepared by partial dissolution of Au-Pt Nanoalloys显示文摘	Marc Schrinner Matthias Ballauff Yaron Kauffmann	2009	Science2009，323，：	1
15	Secondary structure a-nalysis of proteins embedded in spherical polyelectrolyte bru-shes by FT-IR spectroscopy显示文摘	WITTEMANN A BALLAUFF M	2004	Anal Chem2004，76，10：	1
16	Radial density distribution in core-shell latexes as revealed by small-angle X-ray scattering显示文摘	Dingenouts N Kim Y S Ballauff M	1994	Macromolecular Rapid Communications1994，1，7：	1
17	Synthesis of sphericalpolyelectrolyte brushes by photoemulsion polymerization显示文摘	GUO X WEISS A BALLAUFF M	1999	Macromolecules1999，32，19：	1
18	Macromolecules显示文摘	DINGENOUTS N NORHAUSEN Ch BALLAUFF M	1998	(31 ) :8912 - 89171998，，31：	1
19	Observation of the Volume Transition in Thermosensitive Core-Shell Latex Particles by Small-Angle X-Ray Scattering显示文摘	Norhausen Ch Ballauff M	1998	Macromolecules1998，31，：	1
20	Phase equilibria in rod like systems with flexible side chains显示文摘	M Ballauff	1986	Macromolecules1986，19，5：	1

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