116 related articles for article (PubMed ID: 17393522)
1. Bile acid treatment alters hepatic disease and bile acid transport in peroxisome-deficient PEX2 Zellweger mice.
Keane MH; Overmars H; Wikander TM; Ferdinandusse S; Duran M; Wanders RJ; Faust PL
Hepatology; 2007 Apr; 45(4):982-97. PubMed ID: 17393522
[TBL] [Abstract][Full Text] [Related]
2. Disturbed cholesterol homeostasis in a peroxisome-deficient PEX2 knockout mouse model.
Kovacs WJ; Shackelford JE; Tape KN; Richards MJ; Faust PL; Fliesler SJ; Krisans SK
Mol Cell Biol; 2004 Jan; 24(1):1-13. PubMed ID: 14673138
[TBL] [Abstract][Full Text] [Related]
3. The peroxisome deficient PEX2 Zellweger mouse: pathologic and biochemical correlates of lipid dysfunction.
Faust PL; Su HM; Moser A; Moser HW
J Mol Neurosci; 2001; 16(2-3):289-97; discussion 317-21. PubMed ID: 11478384
[TBL] [Abstract][Full Text] [Related]
4. Cholesterol biosynthesis and ER stress in peroxisome deficiency.
Faust PL; Kovacs WJ
Biochimie; 2014 Mar; 98():75-85. PubMed ID: 24211592
[TBL] [Abstract][Full Text] [Related]
5. Mitochondrial alterations caused by defective peroxisomal biogenesis in a mouse model for Zellweger syndrome (PEX5 knockout mouse).
Baumgart E; Vanhorebeek I; Grabenbauer M; Borgers M; Declercq PE; Fahimi HD; Baes M
Am J Pathol; 2001 Oct; 159(4):1477-94. PubMed ID: 11583975
[TBL] [Abstract][Full Text] [Related]
6. Absence of peroxisomes in mouse hepatocytes causes mitochondrial and ER abnormalities.
Dirkx R; Vanhorebeek I; Martens K; Schad A; Grabenbauer M; Fahimi D; Declercq P; Van Veldhoven PP; Baes M
Hepatology; 2005 Apr; 41(4):868-78. PubMed ID: 15732085
[TBL] [Abstract][Full Text] [Related]
7. Targeted deletion of the PEX2 peroxisome assembly gene in mice provides a model for Zellweger syndrome, a human neuronal migration disorder.
Faust PL; Hatten ME
J Cell Biol; 1997 Dec; 139(5):1293-305. PubMed ID: 9382874
[TBL] [Abstract][Full Text] [Related]
8. Role of liver peroxisomes in bile acid formation: inborn error of C27-steroid side chain cleavage in peroxisome deficiency (Zellweger syndrome).
Kase BF
Scand J Clin Lab Invest; 1989 Feb; 49(1):1-10. PubMed ID: 2658011
[TBL] [Abstract][Full Text] [Related]
9. Liver disease caused by failure to racemize trihydroxycholestanoic acid: gene mutation and effect of bile acid therapy.
Setchell KD; Heubi JE; Bove KE; O'Connell NC; Brewsaugh T; Steinberg SJ; Moser A; Squires RH
Gastroenterology; 2003 Jan; 124(1):217-32. PubMed ID: 12512044
[TBL] [Abstract][Full Text] [Related]
10. Bile acid profiles in peroxisomal 3-oxoacyl-coenzyme A thiolase deficiency.
Clayton PT; Patel E; Lawson AM; Carruthers RA; Collins J
J Clin Invest; 1990 Apr; 85(4):1267-73. PubMed ID: 2318981
[TBL] [Abstract][Full Text] [Related]
11. Molecular regulation of sinusoidal liver bile acid transporters during cholestasis.
Gartung C; Matern S
Yale J Biol Med; 1997; 70(4):355-63. PubMed ID: 9626756
[TBL] [Abstract][Full Text] [Related]
12. A novel bile acid biosynthesis defect due to a deficiency of peroxisomal ABCD3.
Ferdinandusse S; Jimenez-Sanchez G; Koster J; Denis S; Van Roermund CW; Silva-Zolezzi I; Moser AB; Visser WF; Gulluoglu M; Durmaz O; Demirkol M; Waterham HR; Gökcay G; Wanders RJ; Valle D
Hum Mol Genet; 2015 Jan; 24(2):361-70. PubMed ID: 25168382
[TBL] [Abstract][Full Text] [Related]
13. Oral bile acid treatment and the patient with Zellweger syndrome.
Setchell KD; Bragetti P; Zimmer-Nechemias L; Daugherty C; Pelli MA; Vaccaro R; Gentili G; Distrutti E; Dozzini G; Morelli A
Hepatology; 1992 Feb; 15(2):198-207. PubMed ID: 1735522
[TBL] [Abstract][Full Text] [Related]
14. Cholic acid therapy in Zellweger spectrum disorders.
Berendse K; Klouwer FC; Koot BG; Kemper EM; Ferdinandusse S; Koelfat KV; Lenicek M; Schaap FG; Waterham HR; Vaz FM; Engelen M; Jansen PL; Wanders RJ; Poll-The BT
J Inherit Metab Dis; 2016 Nov; 39(6):859-868. PubMed ID: 27469511
[TBL] [Abstract][Full Text] [Related]
15. Zellweger syndrome knockout mouse models challenge putative peroxisomal beta-oxidation involvement in docosahexaenoic acid (22:6n-3) biosynthesis.
Infante JP; Huszagh VA
Mol Genet Metab; 2001 Jan; 72(1):1-7. PubMed ID: 11161822
[TBL] [Abstract][Full Text] [Related]
16. Liver disease predominates in a mouse model for mild human Zellweger spectrum disorder.
Berendse K; Boek M; Gijbels M; Van der Wel NN; Klouwer FC; van den Bergh-Weerman MA; Shinde AB; Ofman R; Poll-The BT; Houten SM; Baes M; Wanders RJA; Waterham HR
Biochim Biophys Acta Mol Basis Dis; 2019 Oct; 1865(10):2774-2787. PubMed ID: 31207289
[TBL] [Abstract][Full Text] [Related]
17. Failure of microtubule-mediated peroxisome division and trafficking in disorders with reduced peroxisome abundance.
Nguyen T; Bjorkman J; Paton BC; Crane DI
J Cell Sci; 2006 Feb; 119(Pt 4):636-45. PubMed ID: 16449325
[TBL] [Abstract][Full Text] [Related]
18. PEX11 beta deficiency is lethal and impairs neuronal migration but does not abrogate peroxisome function.
Li X; Baumgart E; Morrell JC; Jimenez-Sanchez G; Valle D; Gould SJ
Mol Cell Biol; 2002 Jun; 22(12):4358-65. PubMed ID: 12024045
[TBL] [Abstract][Full Text] [Related]
19. A mouse genetic model for familial cholestasis caused by ATP8B1 mutations reveals perturbed bile salt homeostasis but no impairment in bile secretion.
Pawlikowska L; Groen A; Eppens EF; Kunne C; Ottenhoff R; Looije N; Knisely AS; Killeen NP; Bull LN; Elferink RP; Freimer NB
Hum Mol Genet; 2004 Apr; 13(8):881-92. PubMed ID: 14976163
[TBL] [Abstract][Full Text] [Related]
20. Determinants of bile formation during development: ontogeny of hepatic bile acid metabolism and transport.
Suchy FJ; Bucuvalas JC; Novak DA
Semin Liver Dis; 1987 May; 7(2):77-84. PubMed ID: 3303347
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]