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Volumn 17, Issue 2, 2016, Pages

Canine models for copper homeostasis disorders

Author keywords

ATP7A; ATP7B; Bedlington terrier; COMMD1; Copper toxicosis; Genetics; Labrador retriever; Menkes disease; Nutrition; Wilson disease

Indexed keywords

COPPER; COPPER ZINC SUPEROXIDE DISMUTASE; GLUTATHIONE; MENKES PROTEIN; METALLOTHIONEIN; PENICILLAMINE; TRIENTINE; WILSON DISEASE PROTEIN; ZINC DERIVATIVE;

EID: 84957559591     PISSN: 16616596     EISSN: 14220067     Source Type: Journal    
DOI: 10.3390/ijms17020196     Document Type: Review
Times cited : (28)

References (115)
  • 1
    • 84866933062 scopus 로고    scopus 로고
    • Copper and zinc, biological role and significance of Copper/Zinc imbalance
    • [CrossRef]
    • Osredkar, J.; Sustar, N. Copper and zinc, biological role and significance of Copper/Zinc imbalance. J. Clin. Toxicol. 2011. [CrossRef]
    • (2011) J. Clin. Toxicol
    • Osredkar, J.1    Sustar, N.2
  • 2
    • 84904643130 scopus 로고    scopus 로고
    • Copper: From neurotransmission to neuroproteostasis
    • [CrossRef] [PubMed]
    • Opazo, C.M.; Greenough, M.A.; Bush, A.I. Copper: From neurotransmission to neuroproteostasis. Front. Aging Neurosci. 2014, 6, 143. [CrossRef] [PubMed]
    • (2014) Front. Aging Neurosci , vol.6 , pp. 143
    • Opazo, C.M.1    Greenough, M.A.2    Bush, A.I.3
  • 3
    • 0018825273 scopus 로고
    • The role of copper in iron metabolism
    • [PubMed]
    • Chan, W.Y.; Rennert, O.M. The role of copper in iron metabolism. Ann. Clin. Lab. Sci. 1980, 10, 338-344. [PubMed]
    • (1980) Ann. Clin. Lab. Sci , vol.10 , pp. 338-344
    • Chan, W.Y.1    Rennert, O.M.2
  • 4
    • 48849113983 scopus 로고    scopus 로고
    • Mitochondrial copper metabolism and delivery to cytochrome C oxidase
    • [CrossRef] [PubMed]
    • Horn, D.; Barrientos, A. Mitochondrial copper metabolism and delivery to cytochrome C oxidase. IUBMB Life 2008, 60, 421-429. [CrossRef] [PubMed]
    • (2008) IUBMB Life , vol.60 , pp. 421-429
    • Horn, D.1    Barrientos, A.2
  • 6
    • 70449710767 scopus 로고    scopus 로고
    • Interactions of peptide amidation and copper: Novel biomarkers and mechanisms of neural dysfunction
    • [CrossRef] [PubMed]
    • Bousquet-Moore, D.; Prohaska, J.R.; Nillni, E.A.; Czyzyk, T.; Wetsel, W.C.; Mains, R.E.; Eipper, B.A. Interactions of peptide amidation and copper: Novel biomarkers and mechanisms of neural dysfunction. Neurobiol. Dis. 2010, 37, 130-140. [CrossRef] [PubMed]
    • (2010) Neurobiol. Dis , vol.37 , pp. 130-140
    • Bousquet-Moore, D.1    Prohaska, J.R.2    Nillni, E.A.3    Czyzyk, T.4    Wetsel, W.C.5    Mains, R.E.6    Eipper, B.A.7
  • 7
    • 0019879833 scopus 로고
    • Roles for iron and copper in connective tissue biosynthesis
    • [CrossRef] [PubMed]
    • O’Dell, B.L. Roles for iron and copper in connective tissue biosynthesis. Philos. Trans. R. Soc. Lond. B. Biol. Sci. 1981, 294, 91-104. [CrossRef] [PubMed]
    • (1981) Philos. Trans. R. Soc. Lond. B. Biol. Sci , vol.294 , pp. 91-104
    • O’Dell, B.L.1
  • 8
    • 78651355486 scopus 로고    scopus 로고
    • ATP7A-related copper transport diseases-emerging concepts and future trends
    • [CrossRef] [PubMed]
    • Kaler, S.G. ATP7A-related copper transport diseases-emerging concepts and future trends. Nat. Rev. Neurol. 2011, 7, 15-29. [CrossRef] [PubMed]
    • (2011) Nat. Rev. Neurol , vol.7 , pp. 15-29
    • Kaler, S.G.1
  • 9
    • 78651124591 scopus 로고
    • A Sex-linked recessive disorder with retardation of growth, peculiar hair, and focal cerebral and cerebellar degeneration
    • [PubMed]
    • Menkes, J.H.; Alter, M.; Steigleder, G.K.; Weakley, D.R.; Sung, J.H. A Sex-linked recessive disorder with retardation of growth, peculiar hair, and focal cerebral and cerebellar degeneration. Pediatrics 1962, 29, 764-779. [PubMed]
    • (1962) Pediatrics , vol.29 , pp. 764-779
    • Menkes, J.H.1    Alter, M.2    Steigleder, G.K.3    Weakley, D.R.4    Sung, J.H.5
  • 10
    • 0027446365 scopus 로고
    • Isolation of a Candidate gene for menkes disease and evidence that it encodes a copper-transporting ATPase
    • [CrossRef] [PubMed]
    • Vulpe, C.; Levinson, B.; Whitney, S.; Packman, S.; Gitschier, J. Isolation of a Candidate gene for menkes disease and evidence that it encodes a copper-transporting ATPase. Nat. Genet. 1993, 3, 7-13. [CrossRef] [PubMed]
    • (1993) Nat. Genet. , vol.3 , pp. 7-13
    • Vulpe, C.1    Levinson, B.2    Whitney, S.3    Packman, S.4    Gitschier, J.5
  • 11
    • 0027452091 scopus 로고
    • TheWilson disease gene is a putative copper transporting P-type ATPase similar to the menkes gene
    • [CrossRef] [PubMed]
    • Bull, P.C.; Thomas, G.R.; Rommens, J.M.; Forbes, J.R.; Cox, D.W. TheWilson disease gene is a putative copper transporting P-type ATPase similar to the menkes gene. Nat. Genet. 1993, 5, 327-337. [CrossRef] [PubMed]
    • (1993) Nat. Genet , vol.5 , pp. 327-337
    • Bull, P.C.1    Thomas, G.R.2    Rommens, J.M.3    Forbes, J.R.4    Cox, D.W.5
  • 13
    • 46249112793 scopus 로고    scopus 로고
    • American association for study of liver diseases (AASLD). Diagnosis and treatment ofWilson disease: An update
    • [CrossRef] [PubMed]
    • Roberts, E.A.; Schilsky, M.L. American association for study of liver diseases (AASLD). Diagnosis and treatment ofWilson disease: An update. Hepatology 2008, 47, 2089-2111. [CrossRef] [PubMed]
    • (2008) Hepatology , vol.47 , pp. 2089-2111
    • Roberts, E.A.1    Schilsky, M.L.2
  • 14
    • 84900010540 scopus 로고    scopus 로고
    • Phenotype-genotype correlations in patients withWilson’s disease
    • [CrossRef] [PubMed]
    • Ferenci, P. Phenotype-genotype correlations in patients withWilson’s disease. Ann. N. Y. Acad. Sci. 2014, 1315, 1-5. [CrossRef] [PubMed]
    • (2014) Ann. N. Y. Acad. Sci , vol.1315 , pp. 1-5
    • Ferenci, P.1
  • 15
    • 0032765624 scopus 로고    scopus 로고
    • Treatment and management ofWilson’s disease
    • [CrossRef] [PubMed]
    • Shimizu, N.; Yamaguchi, Y.; Aoki, T. Treatment and management ofWilson’s disease. Pediatr. Int. 1999, 41, 419-422. [CrossRef] [PubMed]
    • (1999) Pediatr. Int , vol.41 , pp. 419-422
    • Shimizu, N.1    Yamaguchi, Y.2    Aoki, T.3
  • 16
    • 0021179054 scopus 로고
    • Wilson’s disease: Indications for liver transplants
    • [CrossRef] [PubMed]
    • Sternlieb, I. Wilson’s disease: Indications for liver transplants. Hepatology 1984, 4, 15S-17S. [CrossRef] [PubMed]
    • (1984) Hepatology , vol.4 , pp. 15S-17S
    • Sternlieb, I.1
  • 17
    • 0031971699 scopus 로고    scopus 로고
    • Role of Copper in Indian childhood cirrhosis
    • [PubMed]
    • Tanner, M.S. Role of Copper in Indian childhood cirrhosis. Am. J. Clin. Nutr. 1998, 67, 1074S-1081S. [PubMed]
    • (1998) Am. J. Clin. Nutr , vol.67
    • Tanner, M.S.1
  • 18
    • 0029983769 scopus 로고    scopus 로고
    • Endemic tyrolean infantile cirrhosis: An ecogenetic disorder
    • [CrossRef]
    • Muller, T.; Feichtinger, H.; Berger, H.; Muller, W. Endemic tyrolean infantile cirrhosis: An ecogenetic disorder. Lancet 1996, 347, 877-880. [CrossRef]
    • (1996) Lancet , vol.347 , pp. 877-880
    • Muller, T.1    Feichtinger, H.2    Berger, H.3    Muller, W.4
  • 19
    • 0029928263 scopus 로고    scopus 로고
    • Wilson disease and idiopathic copper toxicosis
    • [PubMed]
    • Scheinberg, I.H.; Sternlieb, I. Wilson disease and idiopathic copper toxicosis. Am. J. Clin. Nutr. 1996, 63, 842S-845S. [PubMed]
    • (1996) Am. J. Clin. Nutr , vol.63
    • Scheinberg, I.H.1    Sternlieb, I.2
  • 20
    • 84857358738 scopus 로고    scopus 로고
    • Canine models of copper toxicosis for understanding mammalian copper metabolism
    • [CrossRef] [PubMed]
    • Fieten, H.; Leegwater, P.A.; Watson, A.L.; Rothuizen, J. Canine models of copper toxicosis for understanding mammalian copper metabolism. Mamm. Genome 2012, 23, 62-75. [CrossRef] [PubMed]
    • (2012) Mamm. Genome , vol.23 , pp. 62-75
    • Fieten, H.1    Leegwater, P.A.2    Watson, A.L.3    Rothuizen, J.4
  • 21
    • 0037081771 scopus 로고    scopus 로고
    • Identification of a new copper metabolism gene by positional cloning in a purebred dog population
    • [CrossRef] [PubMed]
    • van De Sluis, B.; Rothuizen, J.; Pearson, P.L.; van Oost, B.A.; Wijmenga, C. Identification of a new copper metabolism gene by positional cloning in a purebred dog population. Hum. Mol. Genet. 2002, 11, 165-173. [CrossRef] [PubMed]
    • (2002) Hum. Mol. Genet , vol.11 , pp. 165-173
    • Van De Sluis, B.1    Rothuizen, J.2    Pearson, P.L.3    Van Oost, B.A.4    Wijmenga, C.5
  • 22
    • 84954501057 scopus 로고    scopus 로고
    • The Menkes and Wilson disease genes counteract in copper toxicosis in labrador retrievers: A new canine model for copper-metabolism disorders
    • [CrossRef] [PubMed]
    • Fieten, H.; Gill, Y.; Martin, A.J.; Concilli, M.; Dirksen, K.; van Steenbeek, F.G.; Spee, B.; van den Ingh, T.S.; Martens, E.C.; Festa, P. The Menkes and Wilson disease genes counteract in copper toxicosis in labrador retrievers: A new canine model for copper-metabolism disorders. Dis. Model. Mech. 2016, 9, 25-38. [CrossRef] [PubMed]
    • (2016) Dis. Model. Mech , vol.9 , pp. 25-38
    • Fieten, H.1    Gill, Y.2    Martin, A.J.3    Concilli, M.4    Dirksen, K.5    Van Steenbeek, F.G.6    Spee, B.7    Van Den Ingh, T.S.8    Martens, E.C.9    Festa, P.10
  • 23
    • 77649224964 scopus 로고    scopus 로고
    • Leading the way: Canine models of genomics and disease
    • [CrossRef]
    • Shearin, A.L.; Ostrander, E.A. Leading the way: Canine models of genomics and disease. Dis. Model. Mech. 2010, 3, 27-34. [CrossRef]
    • (2010) Dis. Model. Mech , vol.3 , pp. 27-34
    • Shearin, A.L.1    Ostrander, E.A.2
  • 25
    • 0025089547 scopus 로고
    • Clinico-pathological studies of LEC rats with hereditary hepatitis and hepatoma in the acute phase of hepatitis
    • [PubMed]
    • Kasai, N.; Osanai, T.; Miyoshi, I.; Kamimura, E.; Yoshida, M.C.; Dempo, K. Clinico-pathological studies of LEC rats with hereditary hepatitis and hepatoma in the acute phase of hepatitis. Lab. Anim. Sci. 1990, 40, 502-505. [PubMed]
    • (1990) Lab. Anim. Sci , vol.40 , pp. 502-505
    • Kasai, N.1    Osanai, T.2    Miyoshi, I.3    Kamimura, E.4    Yoshida, M.C.5    Dempo, K.6
  • 26
    • 0029799960 scopus 로고    scopus 로고
    • The toxic milk mouse is a murine model of wilson disease
    • [CrossRef] [PubMed]
    • Theophilos, M.B.; Cox, D.W.; Mercer, J.F. The toxic milk mouse is a murine model of wilson disease. Hum. Mol. Genet. 1996, 5, 1619-1624. [CrossRef] [PubMed]
    • (1996) Hum. Mol. Genet , vol.5 , pp. 1619-1624
    • Theophilos, M.B.1    Cox, D.W.2    Mercer, J.F.3
  • 28
    • 84865047664 scopus 로고    scopus 로고
    • Conditional knockout of the Menkes disease copper transporter demonstrates its critical role in embryogenesis
    • [CrossRef] [PubMed]
    • Wang, Y.; Zhu, S.; Weisman, G.A.; Gitlin, J.D.; Petris, M.J. Conditional knockout of the Menkes disease copper transporter demonstrates its critical role in embryogenesis. PLoS ONE 2012, 7, e43039. [CrossRef] [PubMed]
    • (2012) Plos ONE , vol.7
    • Wang, Y.1    Zhu, S.2    Weisman, G.A.3    Gitlin, J.D.4    Petris, M.J.5
  • 29
    • 0018600076 scopus 로고
    • A conspectus of research on copper metabolism and requirements of man
    • [PubMed]
    • Mason, K.E. A conspectus of research on copper metabolism and requirements of man. J. Nutr. 1979, 109, 1979-2066. [PubMed]
    • (1979) J. Nutr , vol.109 , pp. 1979-2066
    • Mason, K.E.1
  • 31
    • 74949100486 scopus 로고    scopus 로고
    • New Developments in the regulation of intestinal copper absorption
    • [CrossRef] [PubMed]
    • Van den Berghe, P.V.; Klomp, L.W. New Developments in the regulation of intestinal copper absorption. Nutr. Rev. 2009, 67, 658-672. [CrossRef] [PubMed]
    • (2009) Nutr. Rev , vol.67 , pp. 658-672
    • Van Den Berghe, P.V.1    Klomp, L.W.2
  • 32
    • 0030844529 scopus 로고    scopus 로고
    • HCTR1: A human gene for copper uptake identified by complementation in yeast
    • [CrossRef] [PubMed]
    • Zhou, B.; Gitschier, J. hCTR1: A human gene for copper uptake identified by complementation in yeast. Proc. Natl. Acad. Sci. USA. 1997, 94, 7481-7486. [CrossRef] [PubMed]
    • (1997) Proc. Natl. Acad. Sci. USA , vol.94 , pp. 7481-7486
    • Zhou, B.1    Gitschier, J.2
  • 33
    • 0036240239 scopus 로고    scopus 로고
    • Metallothionein: The multipurpose protein
    • [CrossRef] [PubMed]
    • Coyle, P.; Philcox, J.C.; Carey, L.C.; Rofe, A.M. Metallothionein: The multipurpose protein. Cell. Mol. Life Sci. 2002, 59, 627-647. [CrossRef] [PubMed]
    • (2002) Cell. Mol. Life Sci , vol.59 , pp. 627-647
    • Coyle, P.1    Philcox, J.C.2    Carey, L.C.3    Rofe, A.M.4
  • 34
    • 0024554012 scopus 로고
    • The role of glutathione in copper metabolism and toxicity
    • [PubMed]
    • Freedman, J.H.; Ciriolo, M.R.; Peisach, J. The role of glutathione in copper metabolism and toxicity. J. Biol. Chem. 1989, 264, 5598-5605. [PubMed]
    • (1989) J. Biol. Chem , vol.264 , pp. 5598-5605
    • Freedman, J.H.1    Ciriolo, M.R.2    Peisach, J.3
  • 35
    • 0035117191 scopus 로고    scopus 로고
    • Copper delivery by metallochaperone proteins
    • [CrossRef] [PubMed]
    • Rosenzweig, A.C. Copper delivery by metallochaperone proteins. Acc. Chem. Res. 2001, 34, 119-128. [CrossRef] [PubMed]
    • (2001) Acc. Chem. Res , vol.34 , pp. 119-128
    • Rosenzweig, A.C.1
  • 36
    • 0034721928 scopus 로고    scopus 로고
    • Copper activation of superoxide dismutase 1 (SOD1) in vivo. Role for protein-protein interactions with the copper chaperone for SOD1
    • [CrossRef] [PubMed]
    • Schmidt, P.J.; Kunst, C.; Culotta, V.C. Copper activation of superoxide dismutase 1 (SOD1) in vivo. Role for protein-protein interactions with the copper chaperone for SOD1. J. Biol. Chem. 2000, 275, 33771-33776. [CrossRef] [PubMed]
    • (2000) J. Biol. Chem , vol.275 , pp. 33771-33776
    • Schmidt, P.J.1    Kunst, C.2    Culotta, V.C.3
  • 37
    • 0031045676 scopus 로고    scopus 로고
    • Isolation of a cDNA encoding the human homolog of COX17, a yeast gene essential for mitochondrial copper recruitment. Hum
    • [CrossRef] [PubMed]
    • Amaravadi, R.; Glerum, D.M.; Tzagoloff, A. Isolation of a cDNA encoding the human homolog of COX17, a yeast gene essential for mitochondrial copper recruitment. Hum. Genet. 1997, 99, 329-333. [CrossRef] [PubMed]
    • (1997) Genet , vol.99 , pp. 329-333
    • Amaravadi, R.1    Glerum, D.M.2    Tzagoloff, A.3
  • 38
    • 0030898098 scopus 로고    scopus 로고
    • Identification and functional expression of HAH1, a novel human gene involved in copper homeostasis
    • [PubMed]
    • Klomp, L.W.; Lin, S.J.; Yuan, D.S.; Klausner, R.D.; Culotta, V.C.; Gitlin, J.D. Identification and functional expression of HAH1, a novel human gene involved in copper homeostasis. J. Biol. Chem. 1997, 272, 9221-9226. [PubMed]
    • (1997) J. Biol. Chem , vol.272 , pp. 9221-9226
    • Klomp, L.W.1    Lin, S.J.2    Yuan, D.S.3    Klausner, R.D.4    Culotta, V.C.5    Gitlin, J.D.6
  • 39
    • 84861927586 scopus 로고    scopus 로고
    • Advances in the Understanding of Mammalian Copper Transporters
    • [CrossRef] [PubMed]
    • Wang, Y.; Hodgkinson, V.; Zhu, S.; Weisman, G.A.; Petris, M.J. Advances in the Understanding of Mammalian Copper Transporters. Adv. Nutr. 2011, 2, 129-137. [CrossRef] [PubMed]
    • (2011) Adv. Nutr. , vol.2 , pp. 129-137
    • Wang, Y.1    Hodgkinson, V.2    Zhu, S.3    Weisman, G.A.4    Petris, M.J.5
  • 40
    • 77953851473 scopus 로고    scopus 로고
    • Cardiac copper deficiency activates a systemic signaling mechanism that communicates with the copper acquisition and storage organs
    • [CrossRef] [PubMed]
    • Kim, B.E.; Turski, M.L.; Nose, Y.; Casad, M.; Rockman, H.A.; Thiele, D.J. Cardiac copper deficiency activates a systemic signaling mechanism that communicates with the copper acquisition and storage organs. Cell Metab. 2010, 11, 353-363. [CrossRef] [PubMed]
    • (2010) Cell Metab , vol.11 , pp. 353-363
    • Kim, B.E.1    Turski, M.L.2    Nose, Y.3    Casad, M.4    Rockman, H.A.5    Thiele, D.J.6
  • 42
    • 32044432823 scopus 로고    scopus 로고
    • ATP7B mediates vesicular sequestration of copper: Insight into biliary copper excretion
    • [CrossRef] [PubMed]
    • Cater, M.A.; La Fontaine, S.; Shield, K.; Deal, Y.; Mercer, J.F. ATP7B mediates vesicular sequestration of copper: Insight into biliary copper excretion. Gastroenterology 2006, 130, 493-506. [CrossRef] [PubMed]
    • (2006) Gastroenterology , vol.130 , pp. 493-506
    • Cater, M.A.1    La Fontaine, S.2    Shield, K.3    Deal, Y.4    Mercer, J.F.5
  • 43
    • 34147106917 scopus 로고    scopus 로고
    • Dynamics of endogenous ATP7A (Menkes Protein) in intestinal epithelial cells: Copper-dependent redistribution between two intracellular sites
    • [CrossRef] [PubMed]
    • Nyasae, L.; Bustos, R.; Braiterman, L.; Eipper, B.; Hubbard, A. Dynamics of endogenous ATP7A (Menkes Protein) in intestinal epithelial cells: Copper-dependent redistribution between two intracellular sites. Am. J. Physiol. Gastrointest. Liver Physiol. 2007, 292, G1181-G1194. [CrossRef] [PubMed]
    • (2007) Am. J. Physiol. Gastrointest. Liver Physiol , vol.292
    • Nyasae, L.1    Bustos, R.2    Braiterman, L.3    Eipper, B.4    Hubbard, A.5
  • 46
    • 84856232154 scopus 로고    scopus 로고
    • The copper-transporting capacity of ATP7A mutants associated with menkes disease is ameliorated by COMMD1 as a result of improved protein expression
    • [CrossRef] [PubMed]
    • Vonk, W.I.; de Bie, P.; Wichers, C.G.; van den Berghe, P.V.; van der Plaats, R.; Berger, R.; Wijmenga, C.; Klomp, L.W.; van de Sluis, B. The copper-transporting capacity of ATP7A mutants associated with menkes disease is ameliorated by COMMD1 as a result of improved protein expression. Cell. Mol. Life Sci. 2012, 69, 149-163. [CrossRef] [PubMed]
    • (2012) Cell. Mol. Life Sci , vol.69 , pp. 149-163
    • Vonk, W.I.1    De Bie, P.2    Wichers, C.G.3    Van Den Berghe, P.V.4    Van Der Plaats, R.5    Berger, R.6    Wijmenga, C.7    Klomp, L.W.8    Van De Sluis, B.9
  • 47
    • 34548861803 scopus 로고    scopus 로고
    • Distinct Wilson’s disease mutations in ATP7B are associated with enhanced binding to COMMD1 and reduced stability of ATP7B
    • [CrossRef] [PubMed]
    • De Bie, P.; van de Sluis, B.; Burstein, E.; van de Berghe, P.V.; Muller, P.; Berger, R.; Gitlin, J.D.; Wijmenga, C.; Klomp, L.W. Distinct Wilson’s disease mutations in ATP7B are associated with enhanced binding to COMMD1 and reduced stability of ATP7B. Gastroenterology 2007, 133, 1316-1326. [CrossRef] [PubMed]
    • (2007) Gastroenterology , vol.133 , pp. 1316-1326
    • De Bie, P.1    Van De Sluis, B.2    Burstein, E.3    Van De Berghe, P.V.4    Muller, P.5    Berger, R.6    Gitlin, J.D.7    Wijmenga, C.8    Klomp, L.W.9
  • 48
    • 77954477140 scopus 로고    scopus 로고
    • Roles of COMM-domain-containing 1 in stability and recruitment of the copper-transporting ATPase in a mouse hepatoma cell line
    • [CrossRef] [PubMed]
    • Miyayama, T.; Hiraoka, D.; Kawaji, F.; Nakamura, E.; Suzuki, N.; Ogra, Y. Roles of COMM-domain-containing 1 in stability and recruitment of the copper-transporting ATPase in a mouse hepatoma cell line. Biochem. J. 2010, 429, 53-61. [CrossRef] [PubMed]
    • (2010) Biochem. J. , vol.429 , pp. 53-61
    • Miyayama, T.1    Hiraoka, D.2    Kawaji, F.3    Nakamura, E.4    Suzuki, N.5    Ogra, Y.6
  • 49
    • 67349271590 scopus 로고    scopus 로고
    • Relative efficiencies of plasma catechol levels and ratios for neonatal diagnosis of menkes disease
    • [CrossRef]
    • Goldstein, D.S.; Holmes, C.S.; Kaler, S.G. Relative efficiencies of plasma catechol levels and ratios for neonatal diagnosis of menkes disease. Neurochem. Res. 2009, 34, 1464-1468. [CrossRef]
    • (2009) Neurochem. Res , vol.34 , pp. 1464-1468
    • Goldstein, D.S.1    Holmes, C.S.2    Kaler, S.G.3
  • 51
    • 79958825532 scopus 로고    scopus 로고
    • Phenotypic and genetic characterization of a cohort of pediatricWilson disease patients
    • [CrossRef] [PubMed]
    • Abdel Ghaffar, T.Y.; Elsayed, S.M.; Elnaghy, S.; Shadeed, A.; Elsobky, E.S.; Schmidt, H. Phenotypic and genetic characterization of a cohort of pediatricWilson disease patients. BMC Pediatr. 2011, 11. [CrossRef] [PubMed]
    • (2011) BMC Pediatr , pp. 11
    • Abdel Ghaffar, T.Y.1    Elsayed, S.M.2    Elnaghy, S.3    Shadeed, A.4    Elsobky, E.S.5    Schmidt, H.6
  • 53
    • 33747752509 scopus 로고    scopus 로고
    • Wilson’s disease: An Update
    • [CrossRef] [PubMed]
    • Das, S.K.; Ray, K. Wilson’s disease: An Update. Nat. Clin. Pract. Neurol. 2006, 2, 482-493. [CrossRef] [PubMed]
    • (2006) Nat. Clin. Pract. Neurol , vol.2 , pp. 482-493
    • Das, S.K.1    Ray, K.2
  • 55
    • 0004200257 scopus 로고    scopus 로고
    • accessed on 7 October
    • Wilson Disease Mutation Database. Available online: http://www.Wilsondisease.Med.Ualberta.Ca/Database.Asp (accessed on 7 October 2009).
    • (2009) Wilson Disease Mutation Database
  • 56
    • 67651166903 scopus 로고    scopus 로고
    • Monozygotic female twins discordant for phenotype of Wilson’s disease
    • [CrossRef] [PubMed]
    • Czlonkowska, A.; Gromadzka, G.; Chabik, G. Monozygotic female twins discordant for phenotype of Wilson’s disease. Mov. Disord. 2009, 24, 1066-1069. [CrossRef] [PubMed]
    • (2009) Mov. Disord , vol.24 , pp. 1066-1069
    • Czlonkowska, A.1    Gromadzka, G.2    Chabik, G.3
  • 57
    • 80052920363 scopus 로고    scopus 로고
    • Genetic variability in the methylenetetrahydrofolate reductase gene (MTHFR) affects clinical expression ofWilson’s disease
    • [CrossRef]
    • Gromadzka, G.; Rudnicka, M.; Chabik, G.; Przybylkowski, A.; Czlonkowska, A. Genetic variability in the methylenetetrahydrofolate reductase gene (MTHFR) affects clinical expression ofWilson’s disease. J. Hepatol. 2011, 55, 913-919. [CrossRef]
    • (2011) J. Hepatol , vol.55 , pp. 913-919
    • Gromadzka, G.1    Rudnicka, M.2    Chabik, G.3    Przybylkowski, A.4    Czlonkowska, A.5
  • 58
    • 0033975764 scopus 로고    scopus 로고
    • The impact of apolipoprotein E genotypes on age at onset of symptoms and phenotypic expression in Wilson’s disease
    • [CrossRef] [PubMed]
    • Schiefermeier, M.; Kollegger, H.; Madl, C.; Polli, C.; Oder, W.; Kuhn, H.; Berr, F.; Ferenci, P. The impact of apolipoprotein E genotypes on age at onset of symptoms and phenotypic expression in Wilson’s disease. Brain 2000, 123 Pt 3, 585-590. [CrossRef] [PubMed]
    • (2000) Brain , vol.123 , pp. 585-590
    • Schiefermeier, M.1    Kollegger, H.2    Madl, C.3    Polli, C.4    Oder, W.5    Kuhn, H.6    Berr, F.7    Ferenci, P.8
  • 59
    • 33745825195 scopus 로고    scopus 로고
    • Influence of homozygosity for methionine at codon 129 of the human prion gene on the onset of neurological and hepatic symptoms in Wilson disease
    • [CrossRef]
    • Merle, U.; Stremmel, W.; Gessner, R. Influence of homozygosity for methionine at codon 129 of the human prion gene on the onset of neurological and hepatic symptoms in Wilson disease. Arch. Neurol. 2006, 63, 982-985. [CrossRef]
    • (2006) Arch. Neurol , vol.63 , pp. 982-985
    • Merle, U.1    Stremmel, W.2    Gessner, R.3
  • 60
    • 84860356867 scopus 로고    scopus 로고
    • Apolipoprotein E gene (APOE) genotype inWilson’s disease: Impact on clinical presentation
    • [CrossRef] [PubMed]
    • Litwin, T.; Gromadzka, G.; Czlonkowska, A. Apolipoprotein E gene (APOE) genotype inWilson’s disease: impact on clinical presentation. Parkinsonism Relat. Disord. 2012, 18, 367-369. [CrossRef] [PubMed]
    • (2012) Parkinsonism Relat. Disord. , vol.18 , pp. 367-369
    • Litwin, T.1    Gromadzka, G.2    Czlonkowska, A.3
  • 61
    • 78650588613 scopus 로고    scopus 로고
    • Influence of IL-1RN intron 2 variable number of tandem repeats (VNTR) polymorphism on the age at onset of neuropsychiatric symptoms in Wilson’s disease
    • [CrossRef]
    • Gromadzka, G.; Czlonkowska, A. Influence of IL-1RN intron 2 variable number of tandem repeats (VNTR) polymorphism on the age at onset of neuropsychiatric symptoms in Wilson’s disease. Int. J. Neurosci. 2011, 121, 8-15. [CrossRef]
    • (2011) Int. J. Neurosci , vol.121 , pp. 8-15
    • Gromadzka, G.1    Czlonkowska, A.2
  • 63
    • 0002539614 scopus 로고    scopus 로고
    • Treatment of Wilson’s disease: What are the relative roles of penicillamine, trientine, and zinc supplementation
    • [CrossRef] [PubMed]
    • Schilsky, M.L. Treatment of Wilson’s disease: What are the relative roles of penicillamine, trientine, and zinc supplementation? Curr. Gastroenterol. Rep. 2001, 3, 54-59. [CrossRef] [PubMed]
    • (2001) Curr. Gastroenterol. Rep , vol.3 , pp. 54-59
    • Schilsky, M.L.1
  • 64
    • 84900383927 scopus 로고    scopus 로고
    • D-Penicillamine vs. Zinc sulfate as first-line therapy for Wilson’s disease
    • [CrossRef] [PubMed]
    • Czlonkowska, A.; Litwin, T.; Karlinski, M.; Dziezyc, K.; Chabik, G.; Czerska, M. D-Penicillamine vs. zinc sulfate as first-line therapy for Wilson’s disease. Eur. J. Neurol. 2014, 21, 599-606. [CrossRef] [PubMed]
    • (2014) Eur. J. Neurol , vol.21 , pp. 599-606
    • Czlonkowska, A.1    Litwin, T.2    Karlinski, M.3    Dziezyc, K.4    Chabik, G.5    Czerska, M.6
  • 65
    • 84959175565 scopus 로고    scopus 로고
    • Trientine induced colitis during therapy forWilson disease: A case report and review of the literature
    • [CrossRef] [PubMed]
    • Boga, S.; Jain, D.; Schilsky, M.L. Trientine induced colitis during therapy forWilson disease: A case report and review of the literature. BMC Pharmacol. Toxicol. 2015, 16. 30-015-0031-z. [CrossRef] [PubMed]
    • (2015) BMC Pharmacol. Toxicol , vol.16
    • Boga, S.1    Jain, D.2    Schilsky, M.L.3
  • 66
    • 0029162533 scopus 로고
    • Long-term treatment of Wilson’s disease with Triethylene tetramine dihydrochloride (Trientine)
    • [PubMed]
    • Dahlman, T.; Hartvig, P.; Lofholm, M.; Nordlinder, H.; Loof, L.; Westermark, K. Long-term treatment of Wilson’s disease with Triethylene tetramine dihydrochloride (Trientine). QJM 1995, 88, 609-616. [PubMed]
    • (1995) QJM , vol.88 , pp. 609-616
    • Dahlman, T.1    Hartvig, P.2    Lofholm, M.3    Nordlinder, H.4    Loof, L.5    Westermark, K.6
  • 67
    • 0023178114 scopus 로고
    • Worsening of neurologic syndrome in patients withWilson’s disease with initial Penicillamine therapy
    • [CrossRef] [PubMed]
    • Brewer, G.J.; Terry, C.A.; Aisen, A.M.; Hill, G.M. Worsening of neurologic syndrome in patients withWilson’s disease with initial Penicillamine therapy. Arch. Neurol. 1987, 44, 490-493. [CrossRef] [PubMed]
    • (1987) Arch. Neurol , vol.44 , pp. 490-493
    • Brewer, G.J.1    Terry, C.A.2    Aisen, A.M.3    Hill, G.M.4
  • 68
    • 0028350545 scopus 로고
    • Development of neurologic symptoms in a patient with asymptomatic Wilson’s disease treated with penicillamine
    • [CrossRef] [PubMed]
    • Brewer, G.J.; Turkay, A.; Yuzbaziyan-Gurkan, V. Development of neurologic symptoms in a patient with asymptomatic Wilson’s disease treated with penicillamine. Arch. Neurol. 1994, 51, 304-305. [CrossRef] [PubMed]
    • (1994) Arch. Neurol , vol.51 , pp. 304-305
    • Brewer, G.J.1    Turkay, A.2    Yuzbaziyan-Gurkan, V.3
  • 69
    • 84898457719 scopus 로고    scopus 로고
    • Zinc Monotherapy is effective in Wilson’s disease patients with mild liver disease diagnosed in childhood: A retrospective study
    • [CrossRef]
    • Ranucci, G.; di Dato, F.; Spagnuolo, M.I.; Vajro, P.; Iorio, R. Zinc Monotherapy is effective in Wilson’s disease patients with mild liver disease diagnosed in childhood: a retrospective study. Orphanet J. Rare Dis. 2014, 9. [CrossRef]
    • (2014) Orphanet J. Rare Dis , pp. 9
    • Ranucci, G.1    Di Dato, F.2    Spagnuolo, M.I.3    Vajro, P.4    Iorio, R.5
  • 70
    • 34548666632 scopus 로고    scopus 로고
    • Liver Transplantation in Wilson’s disease: Are its indications established?
    • [CrossRef] [PubMed]
    • Marin, C.; Robles, R.; Parrilla, G.; Ramirez, P.; Bueno, F.S.; Parrilla, P. Liver Transplantation in Wilson’s disease: Are its indications established? Transplant. Proc. 2007, 39, 2300-2301. [CrossRef] [PubMed]
    • (2007) Transplant. Proc. , vol.39 , pp. 2300-2301
    • Marin, C.1    Robles, R.2    Parrilla, G.3    Ramirez, P.4    Bueno, F.S.5    Parrilla, P.6
  • 71
  • 73
    • 0345131727 scopus 로고    scopus 로고
    • Cerebral manifestation of Wilson’s disease successfully treated with liver transplantation
    • [CrossRef] [PubMed]
    • Bax, R.T.; Hassler, A.; Luck, W.; Hefter, H.; Krageloh-Mann, I.; Neuhaus, P.; Emmrich, P. Cerebral manifestation of Wilson’s disease successfully treated with liver transplantation. Neurology 1998, 51, 863-865. [CrossRef] [PubMed]
    • (1998) Neurology , vol.51 , pp. 863-865
    • Bax, R.T.1    Hassler, A.2    Luck, W.3    Hefter, H.4    Krageloh-Mann, I.5    Neuhaus, P.6    Emmrich, P.7
  • 74
    • 2542630457 scopus 로고    scopus 로고
    • Liver transplant team. Liver transplantation for Wilson s disease: Our experience with review of the literature
    • [PubMed]
    • Podgaetz, E.; Chan, C. Liver transplant team. Liver transplantation for Wilson s disease: Our experience with review of the literature. Ann. Hepatol. 2003, 2, 131-134. [PubMed]
    • (2003) Ann. Hepatol , vol.2 , pp. 131-134
    • Podgaetz, E.1    Chan, C.2
  • 75
    • 84879516015 scopus 로고    scopus 로고
    • Indian Childhood Cirrhosis (ICC) & ICC-like diseases: The changing scenario of facts vs. notions
    • [PubMed]
    • Nayak, N.C.; Chitale, A.R. Indian Childhood Cirrhosis (ICC) & ICC-like diseases: The changing scenario of facts vs. notions. Indian J. Med. Res. 2013, 137, 1029-1042. [PubMed]
    • (2013) Indian J. Med. Res , vol.137 , pp. 1029-1042
    • Nayak, N.C.1    Chitale, A.R.2
  • 76
    • 0025892297 scopus 로고
    • Spontaneous hepatic copper accumulation in long-evans cinnamon rats with hereditary hepatitis. A model ofWilson’s disease
    • [CrossRef] [PubMed]
    • Li, Y.; Togashi, Y.; Sato, S.; Emoto, T.; Kang, J.H.; Takeichi, N.; Kobayashi, H.; Kojima, Y.; Une, Y.; Uchino, J. Spontaneous hepatic copper accumulation in long-evans cinnamon rats with hereditary hepatitis. A model ofWilson’s disease. J. Clin. Investig. 1991, 87, 1858-1861. [CrossRef] [PubMed]
    • (1991) J. Clin. Investig , vol.87 , pp. 1858-1861
    • Li, Y.1    Togashi, Y.2    Sato, S.3    Emoto, T.4    Kang, J.H.5    Takeichi, N.6    Kobayashi, H.7    Kojima, Y.8    Une, Y.9    Uchino, J.10
  • 77
    • 0034741120 scopus 로고    scopus 로고
    • Copper-Associated Liver Disease in north ronaldsay sheep: A possible animal model for non-wilsonian hepatic copper toxicosis of infancy and childhood
    • [CrossRef] [PubMed]
    • Haywood, S.; Muller, T.; Muller, W.; Heinz-Erian, P.; Tanner, M.S.; Ross, G. Copper-Associated Liver Disease in north ronaldsay sheep: A possible animal model for non-wilsonian hepatic copper toxicosis of infancy and childhood. J. Pathol. 2001, 195, 264-269. [CrossRef] [PubMed]
    • (2001) J. Pathol , vol.195 , pp. 264-269
    • Haywood, S.1    Muller, T.2    Muller, W.3    Heinz-Erian, P.4    Tanner, M.S.5    Ross, G.6
  • 78
    • 0003823866 scopus 로고
    • 2nd ed.; Clearbrook, B.C., Ed.; Sherpa international, cop.: Dalhousie, QC, Canada
    • Puls, R. Mineral Levels in Animal Health: Diagnostic Data, 2nd ed.; Clearbrook, B.C., Ed.; Sherpa international, cop.: Dalhousie, QC, Canada, 1994.
    • (1994) Mineral Levels in Animal Health: Diagnostic Data
    • Puls, R.1
  • 80
    • 6444238818 scopus 로고    scopus 로고
    • Association between Liver Copper Concentration and subclinical hepatitis in doberman pinschers
    • [CrossRef] [PubMed]
    • Mandigers, P.J.; van den Ingh, T.S.; Bode, P.; Teske, E.; Rothuizen, J. Association between Liver Copper Concentration and subclinical hepatitis in doberman pinschers. J. Vet. Intern. Med. 2004, 18, 647-650. [CrossRef] [PubMed]
    • (2004) J. Vet. Intern. Med , vol.18 , pp. 647-650
    • Mandigers, P.J.1    Van Den Ingh, T.S.2    Bode, P.3    Teske, E.4    Rothuizen, J.5
  • 81
    • 0023883203 scopus 로고
    • Use of 2,3,2-Tetramine as a hepatic copper chelating agent for treatment of copper hepatotoxicosis in bedlington terriers
    • [PubMed]
    • Twedt, D.C.; Hunsaker, H.A.; Allen, K.G. Use of 2,3,2-Tetramine as a hepatic copper chelating agent for treatment of copper hepatotoxicosis in bedlington terriers. J. Am. Vet. Med. Assoc. 1988, 192, 52-56. [PubMed]
    • (1988) J. Am. Vet. Med. Assoc , vol.192 , pp. 52-56
    • Twedt, D.C.1    Hunsaker, H.A.2    Allen, K.G.3
  • 83
    • 0018706646 scopus 로고
    • Clinical, morphologic, and chemical studies on copper toxicosis of bedlington terriers
    • [PubMed]
    • Twedt, D.C.; Sternlieb, I.; Gilbertson, S.R. Clinical, morphologic, and chemical studies on copper toxicosis of bedlington terriers. J. Am. Vet. Med. Assoc. 1979, 175, 269-275. [PubMed]
    • (1979) J. Am. Vet. Med. Assoc , vol.175 , pp. 269-275
    • Twedt, D.C.1    Sternlieb, I.2    Gilbertson, S.R.3
  • 84
    • 3042842753 scopus 로고    scopus 로고
    • Inherited canine copper toxicosis in australian bedlington terriers
    • [PubMed]
    • Hyun, C.; Filippich, L.J. Inherited canine copper toxicosis in australian bedlington terriers. J. Vet. Sci. 2004, 5, 19-28. [PubMed]
    • (2004) J. Vet. Sci , vol.5 , pp. 19-28
    • Hyun, C.1    Filippich, L.J.2
  • 86
    • 0242690424 scopus 로고    scopus 로고
    • The ubiquitously expressed MURR1 protein is absent in canine copper toxicosis
    • [CrossRef]
    • Klomp, A.E.; van de Sluis, B.; Klomp, L.W.; Wijmenga, C. The ubiquitously expressed MURR1 protein is absent in canine copper toxicosis. J. Hepatol. 2003, 39, 703-709. [CrossRef]
    • (2003) J. Hepatol , vol.39 , pp. 703-709
    • Klomp, A.E.1    Van De Sluis, B.2    Klomp, L.W.3    Wijmenga, C.4
  • 87
    • 0020183916 scopus 로고
    • A Comparison of copper-loading disease in bedlington terriers and Wilson’s disease in humans
    • [PubMed]
    • Su, L.C.; Ravanshad, S.; Owen, C.A.; McCall, J.T.; Zollman, P.E.; Hardy, R.M. A Comparison of copper-loading disease in bedlington terriers and Wilson’s disease in humans. Am. J. Physiol. 1982, 243, G226-G230. [PubMed]
    • (1982) Am. J. Physiol , vol.243
    • Su, L.C.1    Ravanshad, S.2    Owen, C.A.3    McCall, J.T.4    Zollman, P.E.5    Hardy, R.M.6
  • 88
    • 80054724654 scopus 로고    scopus 로고
    • Copper-induced hepatitis: The COMMD1 deficient dog as a translational animal model for human chronic hepatitis
    • [CrossRef] [PubMed]
    • Favier, R.P.; Spee, B.; Penning, L.C.; Rothuizen, J. Copper-induced hepatitis: The COMMD1 deficient dog as a translational animal model for human chronic hepatitis. Vet. Q. 2011, 31, 49-60. [CrossRef] [PubMed]
    • (2011) Vet. Q , vol.31 , pp. 49-60
    • Favier, R.P.1    Spee, B.2    Penning, L.C.3    Rothuizen, J.4
  • 89
    • 33748353787 scopus 로고    scopus 로고
    • Copper-associated chronic hepatitis in labrador retrievers
    • [CrossRef] [PubMed]
    • Hoffmann, G.; van den Ingh, T.S.; Bode, P.; Rothuizen, J. Copper-associated chronic hepatitis in labrador retrievers. J. Vet. Intern. Med. 2006, 20, 856-861. [CrossRef] [PubMed]
    • (2006) J. Vet. Intern. Med , vol.20 , pp. 856-861
    • Hoffmann, G.1    Van Den Ingh, T.S.2    Bode, P.3    Rothuizen, J.4
  • 90
    • 33847081260 scopus 로고    scopus 로고
    • Chronic hepatitis in labrador retrievers: Clinical presentation and prognostic factors
    • [CrossRef] [PubMed]
    • Shih, J.L.; Keating, J.H.; Freeman, L.M.; Webster, C.R. Chronic hepatitis in labrador retrievers: Clinical presentation and prognostic factors. J. Vet. Intern. Med. 2007, 21, 33-39. [CrossRef] [PubMed]
    • (2007) J. Vet. Intern. Med , vol.21 , pp. 33-39
    • Shih, J.L.1    Keating, J.H.2    Freeman, L.M.3    Webster, C.R.4
  • 91
    • 67649945902 scopus 로고    scopus 로고
    • Copper-Associated hepatitis in labrador retrievers
    • [CrossRef] [PubMed]
    • Smedley, R.; Mullaney, T.; Rumbeiha, W. Copper-Associated hepatitis in labrador retrievers. Vet. Pathol. 2009, 46, 484-490. [CrossRef] [PubMed]
    • (2009) Vet. Pathol , vol.46 , pp. 484-490
    • Smedley, R.1    Mullaney, T.2    Rumbeiha, W.3
  • 93
    • 0024119371 scopus 로고
    • Hepatitis and copper accumulation in skye terriers
    • [CrossRef] [PubMed]
    • Haywood, S.; Rutgers, H.C.; Christian, M.K. Hepatitis and copper accumulation in skye terriers. Vet. Pathol. 1988, 25, 408-414. [CrossRef] [PubMed]
    • (1988) Vet. Pathol , vol.25 , pp. 408-414
    • Haywood, S.1    Rutgers, H.C.2    Christian, M.K.3
  • 95
    • 0036835637 scopus 로고    scopus 로고
    • Copper-associated liver disease in dalmatians: A review of 10 dogs (1998-2001)
    • Webb, C.B.; Twedt, D.C.; Meyer, D.J. Copper-associated liver disease in dalmatians: A review of 10 dogs (1998-2001). J. Vet. Intern. Med. 2002, 16, 665-668.
    • (2002) J. Vet. Intern. Med , vol.16 , pp. 665-668
    • Webb, C.B.1    Twedt, D.C.2    Meyer, D.J.3
  • 97
    • 49949086849 scopus 로고    scopus 로고
    • Leader of the pack: Gene mapping in dogs and other model organisms
    • [CrossRef] [PubMed]
    • Karlsson, E.K.; Lindblad-Toh, K. Leader of the pack: Gene mapping in dogs and other model organisms. Nat. Rev. Genet. 2008, 9, 713-725. [CrossRef] [PubMed]
    • (2008) Nat. Rev. Genet , vol.9 , pp. 713-725
    • Karlsson, E.K.1    Lindblad-Toh, K.2
  • 99
    • 7944234275 scopus 로고    scopus 로고
    • Improvement in Liver Pathology After 4 Months of D-penicillamine in 5 doberman pinschers with subclinical hepatitis
    • [CrossRef] [PubMed]
    • Mandigers, P.J.; van den Ingh, T.S.; Bode, P.; Rothuizen, J. Improvement in Liver Pathology After 4 Months of D-penicillamine in 5 doberman pinschers with subclinical hepatitis. J. Vet. Intern. Med. 2005, 19, 40-43. [CrossRef] [PubMed]
    • (2005) J. Vet. Intern. Med , vol.19 , pp. 40-43
    • Mandigers, P.J.1    Van Den Ingh, T.S.2    Bode, P.3    Rothuizen, J.4
  • 101
    • 79952706128 scopus 로고    scopus 로고
    • The biogenic methanobactin is an effective chelator for copper in a rat model for wilson disease
    • [CrossRef] [PubMed]
    • Summer, K.H.; Lichtmannegger, J.; Bandow, N.; Choi, D.W.; DiSpirito, A.A.; Michalke, B. The biogenic methanobactin is an effective chelator for copper in a rat model for wilson disease. J. Trace Elem. Med. Biol. 2011, 25, 36-41. [CrossRef] [PubMed]
    • (2011) J. Trace Elem. Med. Biol , vol.25 , pp. 36-41
    • Summer, K.H.1    Lichtmannegger, J.2    Bandow, N.3    Choi, D.W.4    Dispirito, A.A.5    Michalke, B.6
  • 104
    • 84897454220 scopus 로고    scopus 로고
    • Nutritional management of inherited copper-associated hepatitis in the labrador retriever
    • [CrossRef] [PubMed]
    • Fieten, H.; Biourge, V.C.; Watson, A.L.; Leegwater, P.A.; van den Ingh, T.S.; Rothuizen, J. Nutritional management of inherited copper-associated hepatitis in the labrador retriever. Vet. J. 2014, 199, 429-433. [CrossRef] [PubMed]
    • (2014) Vet. J , vol.199 , pp. 429-433
    • Fieten, H.1    Biourge, V.C.2    Watson, A.L.3    Leegwater, P.A.4    Van Den Ingh, T.S.5    Rothuizen, J.6
  • 106
    • 0037428285 scopus 로고    scopus 로고
    • Effects of soy protein isolate on LEC rats, a model of Wilson disease: Mechanisms underlying enhancement of liver cell damage
    • [CrossRef]
    • Yonezawa, K.; Nakagama, H.; Tajima, R.; Ushigome, M.; Ogra, Y.; Suzuki, K.T.; Yoshikawa, K.; Nagao, M. Effects of soy protein isolate on LEC rats, a model of Wilson disease: Mechanisms underlying enhancement of liver cell damage. Biochem. Biophys. Res. Commun. 2003, 302, 271-274. [CrossRef]
    • (2003) Biochem. Biophys. Res. Commun , vol.302 , pp. 271-274
    • Yonezawa, K.1    Nakagama, H.2    Tajima, R.3    Ushigome, M.4    Ogra, Y.5    Suzuki, K.T.6    Yoshikawa, K.7    Nagao, M.8
  • 108
    • 0028057852 scopus 로고
    • Liver transplantation for Wilson’s disease: Indications and outcome
    • [CrossRef] [PubMed]
    • Schilsky, M.L.; Scheinberg, I.H.; Sternlieb, I. Liver transplantation for Wilson’s disease: Indications and outcome. Hepatology 1994, 19, 583-587. [CrossRef] [PubMed]
    • (1994) Hepatology , vol.19 , pp. 583-587
    • Schilsky, M.L.1    Scheinberg, I.H.2    Sternlieb, I.3
  • 114
    • 79751503682 scopus 로고    scopus 로고
    • TALEs of Genome Targeting
    • [CrossRef] [PubMed]
    • Boch, J. TALEs of Genome Targeting. Nat. Biotechnol. 2011, 29, 135-136. [CrossRef] [PubMed]
    • (2011) Nat. Biotechnol , vol.29 , pp. 135-136
    • Boch, J.1
  • 115
    • 74249095519 scopus 로고    scopus 로고
    • CRISPR/Cas, the immune system of bacteria and archaea
    • [CrossRef] [PubMed]
    • Horvath, P.; Barrangou, R. CRISPR/Cas, the immune system of bacteria and archaea. Science 2010, 327, 167-170. [CrossRef] [PubMed]
    • (2010) Science , vol.327 , pp. 167-170
    • Horvath, P.1    Barrangou, R.2


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