Copper incorporation into ceruloplasmin is regulated by Niemann-Pick C1 protein

Hepatology Research

Volumn 41, Issue 5, 2011, Pages 484-491

  Yanagimoto, Chikatoshi ^a,b   Harada, Masaru ^c   Kumemura, Hiroto ^a   Abe, Mitsuhiko ^a   Koga, Hironori ^a   Sakata, Masahiro ^a   Kawaguchi, Takumi ^a   Terada, Kunihiko ^d   Hanada, Shinichiro ^a   Taniguchi, Eitaro ^a   Ninomiya, Haruaki ^f   Ueno, Takato ^a   Sugiyama, Toshihiro ^e   Sata, Michio ^a  

^a KURUME UNIVERSITY SCHOOL OF MEDICINE   (Japan)

^b Yanagimoto naika ^*  (Japan)

^c UNIVERSITY OF OCCUPATIONAL AND ENVIRONMENTAL HEALTH   (Japan)

^d Onoba Hospital   (Japan)

^e AKITA UNIVERSITY SCHOOL OF MEDICINE   (Japan)

^f TOTTORI UNIVERSITY   (Japan)

Author keywords

ATP7B; Ceruloplasmin; Copper; Niemann Pick disease type C; NPC1; Wilson disease

Indexed keywords

ALBUMIN; CERULOPLASMIN; COPPER; GENE PRODUCT; PROTEIN NIEMANN PICK C1; UNCLASSIFIED DRUG; WILSON DISEASE PROTEIN;

ARTICLE; CELL CULTURE; CONTROLLED STUDY; CULTURE MEDIUM; ENDOSOME; GENE OVEREXPRESSION; HUMAN; HUMAN CELL; LIVER CELL; NIEMANN PICK DISEASE; PHENOTYPE; PRIORITY JOURNAL; PROTEIN FUNCTION; PROTEIN LOCALIZATION; PROTEIN SECRETION; SECRETORY VESICLE; TRANS GOLGI NETWORK; TRANSPORT AND BINDING PHENOMENA; WILSON DISEASE;

EID: 79955127682     PISSN: 13866346     EISSN: 1872034X     Source Type: Journal    
DOI: 10.1111/j.1872-034X.2011.00788.x     Document Type: Article

References (36)

1. Linder MC, Hazegh-Azam M. Copper biochemistry and molecular biology. Am J Clin Nutr 1996; 63: 797S-811S.
2. Harada M. Wilson disease. Med Electron Microsc 2002; 35: 61-6.
3. Ala A, Walker AP, Ashkan K, Dooley JS, Schilsky ML. Wilson's disease. Lancet 2007; 369: 397-408.
4. 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.
5. Petrukhin K, Fischer SG, Pirastu M etal. Mapping, cloning and genetic characterization of the region containing the Wilson disease gene. Nat Genet 1993; 5: 338-43.
6. Tanzi RE, Petrukhin K, Chernov I etal. The Wilson disease gene is a copper transporting ATPase with homology to the Menkes disease gene. Nat Genet 1993; 5: 344-50.
7. Yamaguchi Y, Heiny ME, Gitlin JD. Isolation and characterization of a human liver cDNA as a candidate gene forWilson disease. Biochem Biophys Res Commun 1993; 197: 271-7.
8. Terada K, Nakako T, Yang XL etal. Restoration of holoceruloplasmin synthesis in LEC rat after infusion of recombinant adenovirus bearing WND cDNA. J Biol Chem 1998; 273: 1815-20.
9. Terada K, Aiba N, Yang XL etal. Biliary excretion of copper in LEC rat after introduction of copper transporting P-type ATPase, ATP7B. FEBS Lett 1999; 448: 53-6.
10. Harada M, Sakisaka S, Kawaguchi T etal. Copper does not alter the intracellular distribution of ATP7B, a copper-transporting ATPase. Biochem Biophys Res Commun 2000; 275: 871-6.
11. Harada M, Sakisaka S, Terada K etal. Role of ATP7B in biliary copper excretion in a human hepatoma cell line and normal rat hepatocytes. Gastroenterology 2000; 118: 921-8.
12. Harada M, Sakisaka S, Terada K etal. A mutation of the Wilson disease protein, ATP7B, is degraded in the proteasomes and forms protein aggregates. Gastroenterology 2001; 120: 967-74.
13. Harada M, Kumemura H, Sakisaka S etal. Wilson disease protein ATP7B is localized in the late endosomes in a polarized human hepatocyte cell line. Int J Mol Med 2003; 11: 293-8.
14. Harada M, Kawaguchi T, Kumemura H etal. The Wilson disease protein ATP7B resides in the late endosomes with Rab7 and the Niemann-Pick C1 protein. Am J Pathol 2005; 166: 499-510.
15. Yanagimoto C, Harada M, Kumemura H etal. Niemann-Pick C1 protein transports copper to the secretory compartment from late endosomes where ATP7B resides. Exp Cell Res 2009; 15: 119-26.
16. Gross JB Jr, Myers BM, Kost LJ, Kuntz SM, LaRusso NF. Biliary copper excretion by hepatocyte lysosomes in the rat. Major excretory pathway in experimental copper overload. J Clin Invest 1989; 83: 30-9.
17. Harada M, Sakisaka S, Yoshitake M etal. Biliary copper excretion in acutely and chronically copper-loaded rats. Hepatology 1993; 17: 111-7.
18. Terada K, Kawarada Y, Miura N, Yasui O, Koyama K, Sugiyama T. Copper incorporation into ceruloplasmin in rat livers. Biochim Biophys Acta 1995; 1270: 58-62.
19. Griffiths G, Hoflack B, Simons K, Mellman I, Kornfeld S. The mannose 6-phosphate receptor and the biogenesis of lysosomes. Cell 1988; 52: 329-41.
20. Hung IH, Suzuki M, Yamaguchi Y, Yuan DS, Klausner RD, Gitlin JD. Biochemical characterization of the Wilson disease protein and functional expression in the yeast Saccharomyces cerevisiae. J Biol Chem 1997; 272: 21461-6.
21. Yang XL, Miura N, Kawarada Y etal. Two forms of Wilson disease protein produced by alternative splicing are localized in distinct cellular compartments. Biochem J 1997; 326: 897-902.
22. Schaefer M, Hopkins RG, Failla ML, Gitlin JD. Hepatocyte-specific localization and copper-dependent trafficking of the Wilson's disease protein in the liver. Am J Physiol 1999; 276: G639-46.
23. Schaefer M, Roelofsen H, Wolters H etal. Localization of the Wilson's disease protein in human liver. Gastroenterology 1999; 117: 1380-5.
24. Roelofsen H, Wolters H, Van Luyn MJ, Miura N, Kuipers F, Vonk RJ. Copper-induced apical trafficking of ATP7B in polarized hepatoma cells provides a mechanism for biliary copper excretion. Gastroenterology 2000; 119: 782-93.
25. Cater MA, La Fontaine S, Shield K, Deal Y, Mercer JF. ATP7B mediates vesicular sequestration of copper: insight into biliary copper excretion. Gastroenterology 2006; 130: 493-506.
26. Guo Y, Nyasae L, Braiterman LT, Hubbard AL. NH2-terminal signals in ATP7B Cu-ATPase mediate its Cu-dependent anterograde traffic in polarized hepatic cells. Am J Physiol Gastrointest Liver Physiol 2005; 289: G904-16.
27. Carstea ED, Morris JA, Coleman KG etal. Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis. Science 1997; 277: 228-31.
28. Neufeld EB, Wastney M, Patel S etal. The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo. J Biol Chem 1999; 274: 9627-35.
29. Zhang M, Dwyer NK, Neufeld EB etal. Sterol-modulated glycolipid sorting occurs in niemann-pick C1 late endosomes. J Biol Chem 2001; 276: 3417-25.
30. Walter M, Davies JP, Ioannou YA. Telomerase immortalization upregulates Rab9 expression and restores LDL cholesterol egress from Niemann-Pick C1 late endosomes. J Lipid Res 2003; 44: 243-53.
31. Choudhury A, Dominguez M, Puri V etal. Rab proteins mediate Golgi transport of caveola-internalized glycosphingolipids and correct lipid trafficking in Niemann-Pick C cells. J Clin Invest 2002; 109: 1541-50.
32. Ganley IG, Pfeffer SR. Cholesterol accumulation sequesters Rab9 and disrupts late endosome function in NPC1-deficient cells. J Biol Chem 2006; 281: 17890-9.
33. Kobayashi T, Beuchat MH, Lindsay M etal. Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol transport. Nat Cell Biol 1999; 1: 113-8.
34. Chen JW, Murphy TL, Willingham MC, Pastan I, August JT. Identification of two lysosomal membrane glycoproteins. J Cell Biol 1985; 101: 85-95.
35. Wang T, Weinman SA. Involvement of chloride channels in hepatic copper metabolism: ClC-4 promotes copper incorporation into ceruloplasmin. Gastroenterology 2004; 126: 1157-66.
36. Hellman NE, Kono S, Mancini GM, Hoogeboom AJ, De Jong GJ, Gitlin JD. Mechanisms of copper incorporation into human ceruloplasmin. J Biol Chem 2002; 277: 46632-8.