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Items: 1 to 20 of 180

1.

Urinary copper elevation in a mouse model of Wilson's disease is a regulated process to specifically decrease the hepatic copper load.

Gray LW, Peng F, Molloy SA, Pendyala VS, Muchenditsi A, Muzik O, Lee J, Kaplan JH, Lutsenko S.

PLoS One. 2012;7(6):e38327. doi: 10.1371/journal.pone.0038327. Epub 2012 Jun 22.

2.

Positron emission tomography of copper metabolism in the Atp7b-/- knock-out mouse model of Wilson's disease.

Peng F, Lutsenko S, Sun X, Muzik O.

Mol Imaging Biol. 2012 Feb;14(1):70-8. doi: 10.1007/s11307-011-0476-4.

3.

Imaging copper metabolism imbalance in Atp7b (-/-) knockout mouse model of Wilson's disease with PET-CT and orally administered 64CuCl2.

Peng F, Lutsenko S, Sun X, Muzik O.

Mol Imaging Biol. 2012 Oct;14(5):600-7. doi: 10.1007/s11307-011-0532-0.

PMID:
22170165
4.

Fragmentation of mitochondrial cardiolipin by copper ions in the Atp7b-/- mouse model of Wilson's disease.

Yurkova IL, Arnhold J, Fitzl G, Huster D.

Chem Phys Lipids. 2011 Jul;164(5):393-400. doi: 10.1016/j.chemphyslip.2011.05.006. Epub 2011 May 30.

PMID:
21645498
5.

Modifying factors and phenotypic diversity in Wilson's disease.

Lutsenko S.

Ann N Y Acad Sci. 2014 May;1315:56-63. doi: 10.1111/nyas.12420. Epub 2014 Apr 4. Review.

6.

Structural and metabolic changes in Atp7b-/- mouse liver and potential for new interventions in Wilson's disease.

Huster D.

Ann N Y Acad Sci. 2014 May;1315:37-44. doi: 10.1111/nyas.12337. Epub 2014 Feb 12. Review.

PMID:
24697742
7.

Atp7b-/- mice as a model for studies of Wilson's disease.

Lutsenko S.

Biochem Soc Trans. 2008 Dec;36(Pt 6):1233-8. doi: 10.1042/BST0361233. Review.

PMID:
19021531
8.

Early gestational gene transfer with targeted ATP7B expression in the liver improves phenotype in a murine model of Wilson's disease.

Roybal JL, Endo M, Radu A, Gray L, Todorow CA, Zoltick PW, Lutsenko S, Flake AW.

Gene Ther. 2012 Nov;19(11):1085-94. doi: 10.1038/gt.2011.186. Epub 2011 Dec 8.

PMID:
22158007
9.

Laser ablation inductively coupled plasma mass spectrometry imaging of metals in experimental and clinical Wilson's disease.

Boaru SG, Merle U, Uerlings R, Zimmermann A, Flechtenmacher C, Willheim C, Eder E, Ferenci P, Stremmel W, Weiskirchen R.

J Cell Mol Med. 2015 Apr;19(4):806-14. doi: 10.1111/jcmm.12497. Epub 2015 Feb 20.

10.

A systems approach implicates nuclear receptor targeting in the Atp7b(-/-) mouse model of Wilson's disease.

Wilmarth PA, Short KK, Fiehn O, Lutsenko S, David LL, Burkhead JL.

Metallomics. 2012 Jul;4(7):660-8. doi: 10.1039/c2mt20017a. Epub 2012 May 8.

11.

Distinct Wilson's disease mutations in ATP7B are associated with enhanced binding to COMMD1 and reduced stability of ATP7B.

de Bie P, van de Sluis B, Burstein E, van de Berghe PV, Muller P, Berger R, Gitlin JD, Wijmenga C, Klomp LW.

Gastroenterology. 2007 Oct;133(4):1316-26. Epub 2007 Jul 25.

12.

Elevated copper remodels hepatic RNA processing machinery in the mouse model of Wilson's disease.

Burkhead JL, Ralle M, Wilmarth P, David L, Lutsenko S.

J Mol Biol. 2011 Feb 11;406(1):44-58. doi: 10.1016/j.jmb.2010.12.001. Epub 2010 Dec 10.

13.

Cell therapy to remove excess copper in Wilson's disease.

Gupta S.

Ann N Y Acad Sci. 2014 May;1315:70-80. doi: 10.1111/nyas.12450. Review.

14.

Long-term metabolic correction of Wilson's disease in a murine model by gene therapy.

Murillo O, Luqui DM, Gazquez C, Martinez-Espartosa D, Navarro-Blasco I, Monreal JI, Guembe L, Moreno-CermeƱo A, Corrales FJ, Prieto J, Hernandez-Alcoceba R, Gonzalez-Aseguinolaza G.

J Hepatol. 2016 Feb;64(2):419-426. doi: 10.1016/j.jhep.2015.09.014. Epub 2015 Sep 25.

PMID:
26409215
15.

Wilson disease at a single cell level: intracellular copper trafficking activates compartment-specific responses in hepatocytes.

Ralle M, Huster D, Vogt S, Schirrmeister W, Burkhead JL, Capps TR, Gray L, Lai B, Maryon E, Lutsenko S.

J Biol Chem. 2010 Oct 1;285(40):30875-83. doi: 10.1074/jbc.M110.114447. Epub 2010 Jul 20.

16.

Heterozygous tx mice have an increased sensitivity to copper loading: implications for Wilson's disease carriers.

Cheah DM, Deal YJ, Wright PF, Buck NE, Chow CW, Mercer JF, Allen KJ.

Biometals. 2007 Oct;20(5):751-7. Epub 2006 Nov 29.

PMID:
17136311
17.

Hepatic copper-transporting ATPase ATP7B: function and inactivation at the molecular and cellular level.

Bartee MY, Lutsenko S.

Biometals. 2007 Jun;20(3-4):627-37. Epub 2007 Feb 1. Review.

PMID:
17268820
18.

The Long-Evans Cinnamon rat: an animal model for Wilson's disease.

Terada K, Sugiyama T.

Pediatr Int. 1999 Aug;41(4):414-8.

PMID:
10453197
19.

Elevated copper impairs hepatic nuclear receptor function in Wilson's disease.

Wooton-Kee CR, Jain AK, Wagner M, Grusak MA, Finegold MJ, Lutsenko S, Moore DD.

J Clin Invest. 2015 Sep;125(9):3449-60. doi: 10.1172/JCI78991. Epub 2015 Aug 4.

20.

Targeted inactivation of copper transporter Atp7b in hepatocytes causes liver steatosis and obesity in mice.

Muchenditsi A, Yang H, Hamilton JP, Koganti L, Housseau F, Aronov L, Fan H, Pierson H, Bhattacharjee A, Murphy R, Sears C, Potter J, Wooton-Kee CR, Lutsenko S.

Am J Physiol Gastrointest Liver Physiol. 2017 Jul 1;313(1):G39-G49. doi: 10.1152/ajpgi.00312.2016. Epub 2017 Apr 20.

PMID:
28428350

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