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46篇 您的检索式：作者名="Sander AM"

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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	Preinjury emotional and family functioning in caregivers of persons with traumatic brain injury 显示文摘	Sander AM Sherer M Malec JF	2003	Archives of Physical Meclieine and Rehabilitation2003，2，：	1
3	Development of c-Kit-positive cells and the onset of electrical rhythmicity in murine small intestine 显示文摘	Torihashi S Ward AM Sanders KM	1997	Gastroenterology1997，112，1：	1
4	Importance of beta-lacta-mase inhibitor phamacokinetics in the phamaco-dynamics of inhibitor-drug combinations: studies with piperacillin-tazobactam and piperacillin-sulbactam显示文摘	Lister PD Prevan AM Sanders CC	1997	Antimicrob Agents Chemother1997，41，4：	1
5	Age-and gender-specific rate of fractures in Australia:a population-based study显示文摘	Sanders KM Seeman E Ugoni AM	1999	Osteoporos Int1999，88，：	1
6	Early versrs later admission to postancute rehabilitation:impact on functional outcome after traumatic brain injury显示文摘	High WM Roebuck-Spencer T Sander AM	2006	Arch Phys Med Rehabil2006，87，3：	1
7	Chemotherapy for disseminated actinic keratosis with 5-fluorouracil and isotrotinoin显示文摘	Sander CA Pfeiffer C Kligman AM		0，，2：	1
8	Chemotherapy for dissemmated actinic keratosis with 5 -fluorauracil and isotretinoin显示文摘	Pfeiffer C Kligman AM	1997	J Am Acad dermtol1997，36，：	1
9	Glycemic control in young children with diabetes : The role of parental health literacy显示文摘	PULGARN ER SANDERS LM PATIO-FERNANDEZ AM	2014	Patient Educ Couns2014，94，1：	1
10	The unit nurse executive显示文摘	Sanders BH Daridson Am Price SA	1996	Nurs Manage1996，27，1：	1
11	Chemotherapy for disseminated actinic keratosis with 5-fluorouracil and isotretinoin显示文摘	Pfeiffer C Kligman AM	1997	J Am Acad Dermatol1997，36，21：	1
12	Cost-effictiveness of screening for HIV in the era of highly active antiretroviral therapy显示文摘	Sanders GD Bayoumi AM Sundaram V	2005	N Engl J Med2005，352，6：	1
13	A 5-HT receptor in human atrium显示文摘	Kaumann AJ Sanders L Brown AM	1990	Br J Pharmacol1990，100，4：	1
14	Chemotherapy for disseminated actinic keratoses with 5- fluorouracil and isotretinoin 显示文摘	Sander CA Pfeiffer C Kligman AM	1997	J Am Acad Demiatol1997，36，2：	1
15	Solvent effects on the microstructure and properties of 75/25 poly(D,L-lactide-co-glycolide) tissue scaffolds显示文摘	Sander EA Alb AM Nauman EA	2004	J Biomed Mater Res A2004，70，：	1
16	Cost-effectiveness of screening for Hl V in the era of highly active antiretrovirat therapy显示文摘	Sanders GD Ba youmi AM Sundaram V et a1	2005	N Eng J Med2005，352，6：	1
17	Invasive pneumococcal disease and 7-valent pneumococcal conjugate vaccine,the Netherlands显示文摘	Van Deursen AM van Mens SP Sanders EA	2012	Emerg Infect Dis2012，18，11：	1
18	Perceived roles and collaboration between neuropsychologists and speech-language pathologists in rehabilitation显示文摘	Sander AM Raymer A Wertheimer J	2009	Clin Neuropsychol2009，23，7：	1
19	The unit nurse executive显示文摘	Sanders BH Daridson Am Price SA	1996	Nurs Manage1996，27，1：	1
20	Glycemic control in young children with diabetes : The role of parental health literacy显示文摘	PULGRON ER SANDERS LM PATINO-FERNANDEZ AM	2014	Patient Educ Couns2014，94，1：	1

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