Effect of penicillamine and zinc on iron metabolism in Wilson's disease

Scandinavian Journal of Gastroenterology

Volumn 42, Issue 12, 2007, Pages 1495-1500

  Medici, Valentina ^a,b   Leo, Vincenza Di ^a   Lamboglia, Francesca ^a   Bowlus, Christopher L ^b   Tseng, Szu Ching ^b   D'Incà, Renata ^a   Irato, Paola ^a   Burra, Patrizia ^a   Martines, Diego ^a   Sturniolo, Giacomo C ^a  

^a UNIVERSITY OF PADOVA   (Italy)

^b UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

Copper; Hepcidin; Iron; Wilson's disease

Indexed keywords

HEPCIDIN; IRON; PENICILLAMINE; ZINC SULFATE;

ADULT; ANALYTIC METHOD; ARTICLE; CHEMICAL ANALYSIS; CLINICAL ARTICLE; COMPARATIVE STUDY; CONCENTRATION (PARAMETERS); CONTROLLED STUDY; FEMALE; FOLLOW UP; HUMAN; IRON METABOLISM; LIVER BIOPSY; MALE; PRIORITY JOURNAL; VOLUNTEER; WILSON DISEASE;

ADOLESCENT; ADULT; BIOPSY; CHELATING AGENTS; FEMALE; HEPATOLENTICULAR DEGENERATION; HUMANS; IRON; MALE; MIDDLE AGED; PENICILLAMINE; STATISTICS, NONPARAMETRIC; TREATMENT OUTCOME; ZINC SULFATE;

EID: 36248995217     PISSN: 00365521     EISSN: 15027708     Source Type: Journal    
DOI: 10.1080/00365520701514495     Document Type: Article

References (27)

1. Frydman M. Genetic aspects of Wilson's disease. J Gastroenterol Hepatol 1990;5:483-90.
2. Bull PC, Thomas GR, Rommens JM, Forbes JR, Cox DW. The Wilson disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene. Nat Genet 1993;5:327-37.
3. Tanzi RE, Petrukhin K, Chernov I, Pellequer JL, Wasco W, Ross B, et al. The Wilson disease gene is a copper transporting ATPase with homology to the Menkes disease gene. Nat Genet 1993;5:344-50.
4. Kato J, Kobune M, Kohgo Y, Sugawara N, Hisai H, Nakamura T, et al. Hepatic iron deprivation prevents spontaneous development of fulminant hepatitis and liver cancer in Long-Evans Cinnamon rats. J Clin Invest 1996;98:923-9.
5. Hayashi H, Wakusawa S, Yano M. Iron removal by phlebotomy for the prophylaxis of fulminant hepatitis in a Wilson disease model of Long-Evans Cinnamon rats. Hepatol Res 2006;35:276-80.
6. Sargent PJ, Farnaud S, Evans RW. Structure/function overview of proteins involved in iron storage and transport. Curr Med Chem 2005;12:2683-93.
7. Hellman NE, Schaefer M, Gehrke S, Stegen P, Hoffman WJ, Gitlin JD, et al. Hepatic iron overload in aceruloplasminemia. Gut 2000;47:858-60.
8. Schilsky M. The irony of treating Wilson's disease. Am J Gastroenterol 2001;96:3055-7.
9. Ganz T. Hepcidin in iron metabolism. Curr Opin Hematol 2004;11:251-4.
10. Park CH, Valore EV, Warning AJ, Ganz T. Hepcidin, a urinary antimicrobial peptide synthesized in the liver. J Biol Chem 2001;276:7806-10.
11. Gehrke SG, Kulaksiz H, Herrmann T, Riedel HD, Bents K, Veltkamp C, et al. Expression of hepcidin in hereditary hemochromatosis: evidence for a regulation response to the serum transferrin saturation and to non-transferrin-bound iron. Blood 2003;102:371-6.
12. Nicolas G, Viatte L, Lou DQ, Bennoun M, Beaumont C, Kahn A, et al. Constitutive hepcidin expression prevents iron overload in a mouse model of hemochromatosis. Nat Genet 2003;34:97-101.
13. Kulaksiz H, Gehrke SG, Janetzko A, Rost D, Bruckner T, Kallinowski B, et al. Pro-hepcidin: expression and cell specific localisation in the liver and its regulation in hereditary haemochromatosis, chronic renal insufficiency, and renal anaemia. Gut 2004;53:735-43.
14. Nicolas G, Chauvet C, Viatte L, Danan JL, Bigard X, Devaux I, et al. The gene encoding the iron regulatory peptide hepcidin is regulated by anemia, hypoxia, and inflammation. J Clin Invest 2002;110:1037-44.
15. Nicolas G, Bennoun M, Porteu A, Mativet S, Beaumont C, Grandchamp B, et al. Severe iron deficiency anemia in transgenic mice expressing liver hepcidin. Proc Natl Acad Sci USA 2002;99:4596-601.
16. Shiono Y, Wakusawa S, Hayashi H, Takikawa T, Yano M, Okada T, et al. Iron accumulation in the liver of male patients with Wilson's Disease. Am J Gastroenterol 2001;96:3147-51.
17. Walshe JM, Dixon AK. Dangers of non-compliance in Wilson's disease. Lancet 1986;12:845-7.
18. Marcellini M, Di Ciommo V, Callea F, Devito R, Comparcola D, Sartorelli MR, et al. Treatment of Wilson's disease with zinc from the time of diagnosis in pediatric patients: a single-hospital, 10-year follow-up study. J Lab Clin Med 2005;145:139-43.
19. Brewer GJ, Dick RD, Johnson VD, Brunberg JA, Kluin KJ, Fink JK. Treatment of Wilson's disease with zinc: XV long-term follow-up studies. J Lab Clin Med 1998;132:264-78.
20. Sharp P. The molecular basis of copper and iron interactions. Proc Nutr Soc 2004;63:563-9.
21. Nemeth E, Ganz T. Regulation of iron metabolism by hepcidin. Annu Rev Nutr 2006;26:323-42.
22. Aoki CA, Rossaro L, Ramsamooj R, Brandhagen D, Burritt MF, Bowlus CL. Liver hepcidin mRNA correlates with iron stores, but not inflammation, in patients with chronic hepatitis C. J Clin Gastroenterol 2005;39:71-4.
23. Faa G, Nurchi V, Demelia L, Ambu R, Parodo G, Congiu T, et al. Uneven hepatic copper distribution in Wilson's disease. J Hepatol 1995;22:303-8.
24. Tomosugi N, Kawabata H, Wakatabe R, Higuchi M, Yamaya H, Umehara H, et al. Detection of serum hepcidin in renal failure and inflammation by using the ProteinChip System. Blood 2006;108:1381-7.
25. Brookes MJ, Sharma NK, Tselepis C, Iqbal TH. Serum prohepcidin: measuring active hepcidin or a non-functional precursor? Gut 2005;54:169-73.
26. Krause A, Neitz S, Magert HJ, Schulz A, Forssmann WG, Schulz-Knappe P, et al. Leap-1, a novel highly disulfide-bonded human peptide, exhibits antimicrobial activity. FEBS Lett 2000;480:147-50.
27. Merle U, Schaefer M, Ferenci P, Stremmel W. Clinical presentation, diagnosis and long-term outcome of Wilson disease: a cohort study. Gut 2006;56:115-20.