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Year Number of Results
2002 2
2004 2
2006 1
2007 1
2009 1
2013 2
2015 2
2016 2
2017 1
2018 2
2019 3
2020 1
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Jaundice revisited: recent advances in the diagnosis and treatment of inherited cholestatic liver diseases.
Chen HL, Wu SH, Hsu SH, Liou BY, Chen HL, Chang MH. Chen HL, et al. J Biomed Sci. 2018 Oct 26;25(1):75. doi: 10.1186/s12929-018-0475-8. J Biomed Sci. 2018. PMID: 30367658 Free PMC article. Review.
The first three types of PFICs identified (PFIC1, PFIC2, and PFIC3) represent defects in FIC1 (ATP8B1), BSEP (ABCB11), or MDR3 (ABCB4). ...DCDC2 is a newly identified genetic disorder causing neonatal sclerosing cholangitis. Other cholestatic genetic d …
The first three types of PFICs identified (PFIC1, PFIC2, and PFIC3) represent defects in FIC1 (ATP8B1), BSEP (ABCB11), or MDR3 …
Expression Analysis of ATP-Binding Cassette Transporters ABCB11 and ABCB4 in Primary Sclerosing Cholangitis and Variety of Pediatric and Adult Cholestatic and Noncholestatic Liver Diseases.
Thoeni C, Waldherr R, Scheuerer J, Schmitteckert S, Roeth R, Niesler B, Cutz E, Flechtenmacher C, Goeppert B, Schirmacher P, Lasitschka F. Thoeni C, et al. Can J Gastroenterol Hepatol. 2019 Dec 10;2019:1085717. doi: 10.1155/2019/1085717. eCollection 2019. Can J Gastroenterol Hepatol. 2019. PMID: 31886153 Free PMC article.
Loss of functional ABCB11 and ABCB4 proteins causes early-onset refractory cholestasis or cholangiopathy. In this study, we investigated the expression and localization pattern of ABCB11 and ABCB4 using immunohistochemistry and RNA profiling in liver samples from pa …
Loss of functional ABCB11 and ABCB4 proteins causes early-onset refractory cholestasis or cholangiopathy. In this study, we investiga …
BSEP and MDR3 haplotype structure in healthy Caucasians, primary biliary cirrhosis and primary sclerosing cholangitis.
Pauli-Magnus C, Kerb R, Fattinger K, Lang T, Anwald B, Kullak-Ublick GA, Beuers U, Meier PJ. Pauli-Magnus C, et al. Hepatology. 2004 Mar;39(3):779-91. doi: 10.1002/hep.20159. Hepatology. 2004. PMID: 14999697
Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are characterized by a cholestatic pattern of liver damage, also observed in hereditary or acquired dysfunction of the canalicular membrane transporters bile salt export pump (BSEP, A
Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are characterized by a cholestatic pattern of liver d …
Ursodeoxycholic acid in cholestatic liver disease: mechanisms of action and therapeutic use revisited.
Paumgartner G, Beuers U. Paumgartner G, et al. Hepatology. 2002 Sep;36(3):525-31. doi: 10.1053/jhep.2002.36088. Hepatology. 2002. PMID: 12198643 Review.
., bile salt export pump, BSEP, and conjugate export pump, MRP2) into the canalicular membrane of the hepatocyte and, possibly, activation of inserted carriers; (3) protection of hepatocytes against bile acid-induced apoptosis, involving inhibition of mitochondrial membran …
., bile salt export pump, BSEP, and conjugate export pump, MRP2) into the canalicular membrane of the hepatocyte and, possibly, activ …
Identification of novel loci for pediatric cholestatic liver disease defined by KIF12, PPM1F, USP53, LSR, and WDR83OS pathogenic variants.
Maddirevula S, Alhebbi H, Alqahtani A, Algoufi T, Alsaif HS, Ibrahim N, Abdulwahab F, Barr M, Alzaidan H, Almehaideb A, AlSasi O, Alhashem A, Hussaini HA, Wali S, Alkuraya FS. Maddirevula S, et al. Genet Med. 2019 May;21(5):1164-1172. doi: 10.1038/s41436-018-0288-x. Epub 2018 Sep 25. Genet Med. 2019. PMID: 30250217
RESULTS: KIF12, which encodes a microtubule motor protein with a tentative role in cell polarity, was found to harbor three homozygous likely deleterious variants in three families with sclerosing cholangitis. ...In another extended family, we mapped an apparently n …
RESULTS: KIF12, which encodes a microtubule motor protein with a tentative role in cell polarity, was found to harbor three homozygous likel …
Inhibition of intestinal bile acid absorption improves cholestatic liver and bile duct injury in a mouse model of sclerosing cholangitis.
Baghdasaryan A, Fuchs CD, Österreicher CH, Lemberger UJ, Halilbasic E, Påhlman I, Graffner H, Krones E, Fickert P, Wahlström A, Ståhlman M, Paumgartner G, Marschall HU, Trauner M. Baghdasaryan A, et al. J Hepatol. 2016 Mar;64(3):674-81. doi: 10.1016/j.jhep.2015.10.024. Epub 2015 Oct 31. J Hepatol. 2016. PMID: 26529078 Free article.
METHODS: Eight week old Mdr2(-/-) (Abcb4(-/-)) mice (model of cholestatic liver injury and sclerosing cholangitis) received either a diet supplemented with A4250 (0.01% w/w) - a highly potent and selective ASBT inhibitor - or a chow diet. ...RESULTS: A4250 improved …
METHODS: Eight week old Mdr2(-/-) (Abcb4(-/-)) mice (model of cholestatic liver injury and sclerosing cholangitis) received ei …
Protein Abundance of Hepatic Drug Transporters in Patients With Different Forms of Liver Damage.
Drozdzik M, Szelag-Pieniek S, Post M, Zeair S, Wrzesinski M, Kurzawski M, Prieto J, Oswald S. Drozdzik M, et al. Clin Pharmacol Ther. 2020 May;107(5):1138-1148. doi: 10.1002/cpt.1717. Epub 2019 Dec 17. Clin Pharmacol Ther. 2020. PMID: 31697849
Liver function deterioration (Child-Pugh class C) produced significant protein abundance (mean values) increase (to healthy livers) in P-gp (to 260% (CV (coefficient of variation) 82%)) and MRP4 (CV 230%) (not detected in healthy livers), decrease in MRP2 (to 30% (CV 126%)), NTCP …
Liver function deterioration (Child-Pugh class C) produced significant protein abundance (mean values) increase (to healthy livers) in P-gp …
A new xenobiotic-induced mouse model of sclerosing cholangitis and biliary fibrosis.
Fickert P, Stöger U, Fuchsbichler A, Moustafa T, Marschall HU, Weiglein AH, Tsybrovskyy O, Jaeschke H, Zatloukal K, Denk H, Trauner M. Fickert P, et al. Am J Pathol. 2007 Aug;171(2):525-36. doi: 10.2353/ajpath.2007.061133. Epub 2007 Jun 28. Am J Pathol. 2007. PMID: 17600122 Free PMC article.
Expression of Ntcp, Oatp4, and Mrp2 was significantly reduced, whereas Bsep expression remained unchanged and adaptive Mrp3 and Mrp4 expression was significantly induced. ...
Expression of Ntcp, Oatp4, and Mrp2 was significantly reduced, whereas Bsep expression remained unchanged and adaptive Mrp3 and Mrp4 …
Cholestatic liver disease.
Jüngst C, Lammert F. Jüngst C, et al. Dig Dis. 2013;31(1):152-4. doi: 10.1159/000347210. Epub 2013 Jun 17. Dig Dis. 2013. PMID: 23797137 Review.
Chronic cholestatic diseases are mostly intrahepatic with the exception of primary and secondary sclerosing cholangitis affecting intra- and extrahepatic bile ducts. Recent genome-wide association studies have confirmed major histocompatibility complex associations …
Chronic cholestatic diseases are mostly intrahepatic with the exception of primary and secondary sclerosing cholangitis affect …
The genetics of complex cholestatic disorders.
Hirschfield GM, Chapman RW, Karlsen TH, Lammert F, Lazaridis KN, Mason AL. Hirschfield GM, et al. Gastroenterology. 2013 Jun;144(7):1357-74. doi: 10.1053/j.gastro.2013.03.053. Epub 2013 Apr 10. Gastroenterology. 2013. PMID: 23583734 Free PMC article. Review.
However, recent genome-wide studies have provided insight into the pathogenesis of gallstones, primary biliary cirrhosis, and primary sclerosing cholangitis. ...In contrast, studies have associated primary biliary cirrhosis and primary sclerosing cholangit
However, recent genome-wide studies have provided insight into the pathogenesis of gallstones, primary biliary cirrhosis, and primary scl
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