-
2
-
-
69749100311
-
Wilson disease: Current status and the future
-
Schilsky ML (2009) Wilson disease: current status and the future. Biochimie 91: 1278-1281.
-
(2009)
Biochimie
, vol.91
, pp. 1278-1281
-
-
Schilsky, M.L.1
-
3
-
-
34250163579
-
Perspectives for gene therapy of Wilson disease
-
Merle U, Stremmel W, Encke J (2007) Perspectives for gene therapy of Wilson disease. Curr Gene Ther 7: 217-220.
-
(2007)
Curr Gene Ther
, vol.7
, pp. 217-220
-
-
Merle, U.1
Stremmel, W.2
Encke, J.3
-
4
-
-
25144471713
-
Liver transplantation for Wilson's disease: The burden of neurological and psychiatric disorders
-
Medici V, Mirante VG, Fassati LR, Pompili M, Forti D, et al. (2005) Liver transplantation for Wilson's disease: The burden of neurological and psychiatric disorders. Liver Transpl 11: 1056-1063.
-
(2005)
Liver Transpl
, vol.11
, pp. 1056-1063
-
-
Medici, V.1
Mirante, V.G.2
Fassati, L.R.3
Pompili, M.4
Forti, D.5
-
5
-
-
37049036616
-
Sequence variation database for the Wilson disease copper transporter, ATP7B
-
Kenney SM, Cox DW (2007) Sequence variation database for the Wilson disease copper transporter, ATP7B. Hum Mutat 28: 1171-1177.
-
(2007)
Hum Mutat
, vol.28
, pp. 1171-1177
-
-
Kenney, S.M.1
Cox, D.W.2
-
6
-
-
80053648012
-
Relative exchangeable copper: A new highly sensitive and highly specific biomarker for Wilson's disease diagnosis
-
El Balkhi S, Trocello JM, Poupon J, Chappuis P, Massicot F, et al. (2011) Relative exchangeable copper: a new highly sensitive and highly specific biomarker for Wilson's disease diagnosis. Clin Chim Acta 412: 2254-2260.
-
(2011)
Clin Chim Acta
, vol.412
, pp. 2254-2260
-
-
El Balkhi, S.1
Trocello, J.M.2
Poupon, J.3
Chappuis, P.4
Massicot, F.5
-
7
-
-
34247897218
-
Stable isotope pilot study of exchangeable copper kinetics in human blood plasma
-
Venelinov TI, Beattie JH, Dainty JR, Hollands WJ, Fairweather-Tait SJ, et al. (2007) Stable isotope pilot study of exchangeable copper kinetics in human blood plasma. J Trace Elem Med Biol 21: 138-140.
-
(2007)
J Trace Elem Med Biol
, vol.21
, pp. 138-140
-
-
Venelinov, T.I.1
Beattie, J.H.2
Dainty, J.R.3
Hollands, W.J.4
Fairweather-Tait, S.J.5
-
8
-
-
55149086561
-
Analytical variables affecting exchangeable copper determination in blood plasma
-
Buckley WT, Vanderpool RA (2008) Analytical variables affecting exchangeable copper determination in blood plasma. Biometals 21: 601-612.
-
(2008)
Biometals
, vol.21
, pp. 601-612
-
-
Buckley, W.T.1
Vanderpool, R.A.2
-
9
-
-
77955644581
-
Human plasma copper proteins speciation by size exclusion chromatography coupled to inductively coupled plasma mass spectrometry. Solutions for columns calibration by sulfur detection
-
El Balkhi S, Poupon J, Trocello JM, Massicot F, Woimant F, et al. (2010) Human plasma copper proteins speciation by size exclusion chromatography coupled to inductively coupled plasma mass spectrometry. Solutions for columns calibration by sulfur detection. Anal Chem 82: 6904-6910.
-
(2010)
Anal Chem
, vol.82
, pp. 6904-6910
-
-
El Balkhi, S.1
Poupon, J.2
Trocello, J.M.3
Massicot, F.4
Woimant, F.5
-
10
-
-
84879190000
-
Genetically confirmed Wilson disease in a 9-month old boy with elevations of aminotransferases
-
Kim JW, Kim JH, Seo JK, Ko JS, Chang JY, et al. (2013) Genetically confirmed Wilson disease in a 9-month old boy with elevations of aminotransferases. World J Hepatol 5: 156-159.
-
(2013)
World J Hepatol
, vol.5
, pp. 156-159
-
-
Kim, J.W.1
Kim, J.H.2
Seo, J.K.3
Ko, J.S.4
Chang, J.Y.5
-
11
-
-
33847137369
-
Late neurological presentations of Wilson disease patients in French population and identification of 8 novel mutations in the ATP7B gene
-
Chappuis P, Callebert J, Quignon V, Woimant F, Laplanche JL (2007) Late neurological presentations of Wilson disease patients in French population and identification of 8 novel mutations in the ATP7B gene. J Trace Elem Med Biol 21: 37-42.
-
(2007)
J Trace Elem Med Biol
, vol.21
, pp. 37-42
-
-
Chappuis, P.1
Callebert, J.2
Quignon, V.3
Woimant, F.4
Laplanche, J.L.5
-
12
-
-
51749083745
-
Relevance of animal models for understanding mammalian copper homeostasis
-
Vonk WI, Wijmenga C, van de Sluis B (2008) Relevance of animal models for understanding mammalian copper homeostasis. Am J Clin Nutr 88: 840S-845S.
-
(2008)
Am J Clin Nutr
, vol.88
-
-
Vonk, W.I.1
Wijmenga, C.2
Van De Sluis, B.3
-
13
-
-
0023550725
-
New mutation causing hereditary hepatitis in the laboratory rat
-
Yoshida MC, Masuda R, Sasaki M, Takeichi N, Kobayashi H, et al. (1987) New mutation causing hereditary hepatitis in the laboratory rat. J Hered 78: 361-365.
-
(1987)
J Hered
, vol.78
, pp. 361-365
-
-
Yoshida, M.C.1
Masuda, R.2
Sasaki, M.3
Takeichi, N.4
Kobayashi, H.5
-
14
-
-
0028965821
-
Copper metabolism leading to and following acute hepatitis in LEC rats
-
Suzuki KT, Kanno S, Misawa S, Aoki Y (1995) Copper metabolism leading to and following acute hepatitis in LEC rats. Toxicology 97: 81-92.
-
(1995)
Toxicology
, vol.97
, pp. 81-92
-
-
Suzuki, K.T.1
Kanno, S.2
Misawa, S.3
Aoki, Y.4
-
15
-
-
0023988973
-
Hereditary hepatitis of LEC rats is controlled by a single autosomal recessive gene
-
Masuda R, Yoshida MC, Sasaki M, Dempo K, Mori M (1988) Hereditary hepatitis of LEC rats is controlled by a single autosomal recessive gene. Lab Anim 22: 166-169.
-
(1988)
Lab Anim
, vol.22
, pp. 166-169
-
-
Masuda, R.1
Yoshida, M.C.2
Sasaki, M.3
Dempo, K.4
Mori, M.5
-
16
-
-
33751008834
-
Accumulation of copper induces DNA strand breaks in brain cells of Long-Evans Cinnamon (LEC) rats, an animal model for human Wilson Disease
-
Hayashi M, Fuse S, Endoh D, Horiguchi N, Nakayama K, et al. (2006) Accumulation of copper induces DNA strand breaks in brain cells of Long-Evans Cinnamon (LEC) rats, an animal model for human Wilson Disease. Exp Anim 55: 419-426.
-
(2006)
Exp Anim
, vol.55
, pp. 419-426
-
-
Hayashi, M.1
Fuse, S.2
Endoh, D.3
Horiguchi, N.4
Nakayama, K.5
-
17
-
-
0028001088
-
The LEC rat has a deletion in the copper transporting ATPase gene homologous to the Wilson disease gene
-
Wu J, Forbes JR, Chen HS, Cox DW (1994) The LEC rat has a deletion in the copper transporting ATPase gene homologous to the Wilson disease gene. Nat Genet 7: 541-545.
-
(1994)
Nat Genet
, vol.7
, pp. 541-545
-
-
Wu, J.1
Forbes, J.R.2
Chen, H.S.3
Cox, D.W.4
-
18
-
-
84873927802
-
Dietary copper triggers onset of fulminant hepatitis in the Long-Evans cinnamon rat model
-
Siaj R, Sauer V, Stoppeler S, Spiegel HU, Kohler G, et al. (2013) Dietary copper triggers onset of fulminant hepatitis in the Long-Evans cinnamon rat model. World J Gastroenterol 18: 5542-5550.
-
(2013)
World J Gastroenterol
, vol.18
, pp. 5542-5550
-
-
Siaj, R.1
Sauer, V.2
Stoppeler, S.3
Spiegel, H.U.4
Kohler, G.5
-
19
-
-
0025892297
-
Spontaneous hepatic copper accumulation in Long-Evans Cinnamon rats with hereditary hepatitis. A model of Wilson's disease
-
Li Y, Togashi Y, Sato S, Emoto T, Kang JH, et al. (1991) Spontaneous hepatic copper accumulation in Long-Evans Cinnamon rats with hereditary hepatitis. A model of Wilson's disease. J Clin Invest 87: 1858-1861.
-
(1991)
J Clin Invest
, vol.87
, pp. 1858-1861
-
-
Li, Y.1
Togashi, Y.2
Sato, S.3
Emoto, T.4
Kang, J.H.5
-
20
-
-
85045533444
-
Inhibition of hereditary hepatitis and liver tumor development in Long-Evans cinnamon rats by the copper-chelating agent trientine dihydrochloride
-
Sone K, Maeda M, Wakabayashi K, Takeichi N, Mori M, et al. (1996) Inhibition of hereditary hepatitis and liver tumor development in Long-Evans cinnamon rats by the copper-chelating agent trientine dihydrochloride. Hepatology 23: 764-770.
-
(1996)
Hepatology
, vol.23
, pp. 764-770
-
-
Sone, K.1
Maeda, M.2
Wakabayashi, K.3
Takeichi, N.4
Mori, M.5
-
21
-
-
5144232782
-
Role of tumor necrosis factor-alpha in the development of spontaneous hepatic toxicity in Long-Evans Cinnamon rats
-
Fong RN, Gonzalez BP, Fuentealba IC, Cherian MG (2004) Role of tumor necrosis factor-alpha in the development of spontaneous hepatic toxicity in Long-Evans Cinnamon rats. Toxicol Appl Pharmacol 200: 121-130.
-
(2004)
Toxicol Appl Pharmacol
, vol.200
, pp. 121-130
-
-
Fong, R.N.1
Gonzalez, B.P.2
Fuentealba, I.C.3
Cherian, M.G.4
-
22
-
-
0034570001
-
Recognizing pain and distress in laboratory animals
-
Carstens E, Moberg GP (2000) Recognizing pain and distress in laboratory animals. ILAR J 41: 62-71.
-
(2000)
ILAR J
, vol.41
, pp. 62-71
-
-
Carstens, E.1
Moberg, G.P.2
-
23
-
-
70349748259
-
Serum ceruloplasmin oxidase activity is a sensitive and highly specific diagnostic marker for Wilson's disease
-
Merle U, Eisenbach C, Weiss KH, Tuma S, Stremmel W (2009) Serum ceruloplasmin oxidase activity is a sensitive and highly specific diagnostic marker for Wilson's disease. J Hepatol 51: 925-930.
-
(2009)
J Hepatol
, vol.51
, pp. 925-930
-
-
Merle, U.1
Eisenbach, C.2
Weiss, K.H.3
Tuma, S.4
Stremmel, W.5
-
24
-
-
0016331976
-
Measurement of ceruloplasmin from its oxidase activity in serum by use of o-dianisidine dihydrochloride
-
Schosinsky KH, Lehmann HP, Beeler MF (1974) Measurement of ceruloplasmin from its oxidase activity in serum by use of o-dianisidine dihydrochloride. Clin Chem 20: 1556-1563.
-
(1974)
Clin Chem
, vol.20
, pp. 1556-1563
-
-
Schosinsky, K.H.1
Lehmann, H.P.2
Beeler, M.F.3
-
25
-
-
67449110713
-
Determination of ultrafiltrable and exchangeable copper in plasma: Stability and reference values in healthy subjects
-
El Balkhi S, Poupon J, Trocello JM, Leyendecker A, Massicot F, et al. (2009) Determination of ultrafiltrable and exchangeable copper in plasma: stability and reference values in healthy subjects. Anal Bioanal Chem 394: 1477-1484.
-
(2009)
Anal Bioanal Chem
, vol.394
, pp. 1477-1484
-
-
El Balkhi, S.1
Poupon, J.2
Trocello, J.M.3
Leyendecker, A.4
Massicot, F.5
-
26
-
-
17144458094
-
Restoration of holoceruloplasmin synthesis in LEC rat after infusion of recombinant adenovirus bearing WND cDNA
-
Terada K, Nakako T, Yang XL, Iida M, Aiba N, et al. (1998) Restoration of holoceruloplasmin synthesis in LEC rat after infusion of recombinant adenovirus bearing WND cDNA. J Biol Chem 273: 1815-1820.
-
(1998)
J Biol Chem
, vol.273
, pp. 1815-1820
-
-
Terada, K.1
Nakako, T.2
Yang, X.L.3
Iida, M.4
Aiba, N.5
-
27
-
-
0025089547
-
Clinicopathological studies of LEC rats with hereditary hepatitis and hepatoma in the acute phase of hepatitis
-
Kasai N, Osanai T, Miyoshi I, Kamimura E, Yoshida MC, et al. (1990) Clinicopathological studies of LEC rats with hereditary hepatitis and hepatoma in the acute phase of hepatitis. Lab Anim Sci 40: 502-505.
-
(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
-
28
-
-
34249082446
-
Hepatic copper-transporting ATPase ATP7B: Function and inactivation at the molecular and cellular level
-
Bartee MY, Lutsenko S (2007) Hepatic copper-transporting ATPase ATP7B: function and inactivation at the molecular and cellular level. Biometals 20: 627-637.
-
(2007)
Biometals
, vol.20
, pp. 627-637
-
-
Bartee, M.Y.1
Lutsenko, S.2
-
29
-
-
84862649408
-
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, et al. (2012) Urinary copper elevation in a mouse model of Wilson's disease is a regulated process to specifically decrease the hepatic copper load. PLoS One 7: e38327.
-
(2012)
PLoS One
, vol.7
-
-
Gray, L.W.1
Peng, F.2
Molloy, S.A.3
Pendyala, V.S.4
Muchenditsi, A.5
-
30
-
-
0026757031
-
Treatment of Wilson's disease with zinc: X. Intestinal metallothionein induction
-
Yuzbasiyan-Gurkan V, Grider A, Nostrant T, Cousins RJ, Brewer GJ (1992) Treatment of Wilson's disease with zinc: X. Intestinal metallothionein induction. J Lab Clin Med 120: 380-386.
-
(1992)
J Lab Clin Med
, vol.120
, pp. 380-386
-
-
Yuzbasiyan-Gurkan, V.1
Grider, A.2
Nostrant, T.3
Cousins, R.J.4
Brewer, G.J.5
-
32
-
-
84861912162
-
PET with 64Cu-histidine for noninvasive diagnosis of biliary copper excretion in Long-Evans cinnamon rat model of Wilson disease
-
Bahde R, Kapoor S, Bhargava KK, Schilsky ML, Palestro CJ, et al. (2012) PET with 64Cu-histidine for noninvasive diagnosis of biliary copper excretion in Long-Evans cinnamon rat model of Wilson disease. J Nucl Med 53: 961-968.
-
(2012)
J Nucl Med
, vol.53
, pp. 961-968
-
-
Bahde, R.1
Kapoor, S.2
Bhargava, K.K.3
Schilsky, M.L.4
Palestro, C.J.5
-
33
-
-
7044260968
-
Restoration of copper metabolism and rescue of hepatic abnormalities in LEC rats, an animal model of Wilson disease, by expression of human ATP7B gene
-
Meng Y, Miyoshi I, Hirabayashi M, Su M, Mototani Y, et al. (2004) Restoration of copper metabolism and rescue of hepatic abnormalities in LEC rats, an animal model of Wilson disease, by expression of human ATP7B gene. Biochim Biophys Acta 1690: 208-219.
-
(2004)
Biochim Biophys Acta
, vol.1690
, pp. 208-219
-
-
Meng, Y.1
Miyoshi, I.2
Hirabayashi, M.3
Su, M.4
Mototani, Y.5
|