-
1
-
-
84918590638
-
Wilson's disease and other neurological copper disorders
-
O. Bandmann, K.H. Weiss, and S.G. Kaler Wilson's disease and other neurological copper disorders Lancet Neurol 14 2015 103 113
-
(2015)
Lancet Neurol
, vol.14
, pp. 103-113
-
-
Bandmann, O.1
Weiss, K.H.2
Kaler, S.G.3
-
2
-
-
0036400025
-
Ceruloplasmin metabolism and function
-
N.E. Hellman, and J.D. Gitlin Ceruloplasmin metabolism and function Annu Rev Nutr 22 2002 439 458
-
(2002)
Annu Rev Nutr
, vol.22
, pp. 439-458
-
-
Hellman, N.E.1
Gitlin, J.D.2
-
3
-
-
36448983237
-
Molecular pathogenesis of Wilson and Menkes disease: Correlation of mutations with molecular defects and disease phenotypes
-
P. de Bie, P. Muller, C. Wijmenga, and L.W. Klomp Molecular pathogenesis of Wilson and Menkes disease: correlation of mutations with molecular defects and disease phenotypes J Med Genet 44 2007 673 688
-
(2007)
J Med Genet
, vol.44
, pp. 673-688
-
-
De Bie, P.1
Muller, P.2
Wijmenga, C.3
Klomp, L.W.4
-
4
-
-
84857633568
-
Evolving perspectives in Wilson disease: Diagnosis, treatment and monitoring
-
K.H. Weiss, and W. Stremmel Evolving perspectives in Wilson disease: diagnosis, treatment and monitoring Curr Gastroenterol Rep 14 2012 1 7
-
(2012)
Curr Gastroenterol Rep
, vol.14
, pp. 1-7
-
-
Weiss, K.H.1
Stremmel, W.2
-
5
-
-
84899996640
-
Clinical considerations for an effective medical therapy in Wilson's disease
-
K.H. Weiss, and W. Stremmel Clinical considerations for an effective medical therapy in Wilson's disease Ann N Y Acad Sci 1315 2014 81 85
-
(2014)
Ann N y Acad Sci
, vol.1315
, pp. 81-85
-
-
Weiss, K.H.1
Stremmel, W.2
-
6
-
-
84897411951
-
Worsening of Wilson disease following penicillamine therapy
-
J. Kalita, V. Kumar, S. Chandra, B. Kumar, and U.K. Misra Worsening of Wilson disease following penicillamine therapy Eur Neurol 71 2014 126 131
-
(2014)
Eur Neurol
, vol.71
, pp. 126-131
-
-
Kalita, J.1
Kumar, V.2
Chandra, S.3
Kumar, B.4
Misra, U.K.5
-
7
-
-
84896064958
-
Long-term outcomes of patients with Wilson disease in a large Austrian cohort
-
S. Beinhardt, W. Leiss, A.F. Stattermayer, I. Graziadei, H. Zoller, R. Stauber, and et al. Long-term outcomes of patients with Wilson disease in a large Austrian cohort Clin Gastroenterol Hepatol 12 2014 683 689
-
(2014)
Clin Gastroenterol Hepatol
, vol.12
, pp. 683-689
-
-
Beinhardt, S.1
Leiss, W.2
Stattermayer, A.F.3
Graziadei, I.4
Zoller, H.5
Stauber, R.6
-
8
-
-
0036158844
-
Early cell transplantation in LEC rats modeling Wilson's disease eliminates hepatic copper with reversal of liver disease
-
H. Malhi, A.N. Irani, I. Volenberg, M.L. Schilsky, and S. Gupta Early cell transplantation in LEC rats modeling Wilson's disease eliminates hepatic copper with reversal of liver disease Gastroenterology 122 2002 438 447
-
(2002)
Gastroenterology
, vol.122
, pp. 438-447
-
-
Malhi, H.1
Irani, A.N.2
Volenberg, I.3
Schilsky, M.L.4
Gupta, S.5
-
9
-
-
0032878550
-
Null mutation of the murine ATP7B (Wilson disease) gene results in intracellular copper accumulation and late-onset hepatic nodular transformation
-
O.I. Buiakova, J. Xu, S. Lutsenko, S. Zeitlin, K. Das, S. Das, and et al. Null mutation of the murine ATP7B (Wilson disease) gene results in intracellular copper accumulation and late-onset hepatic nodular transformation Hum Mol Genet 8 1999 1665 1671
-
(1999)
Hum Mol Genet
, vol.8
, pp. 1665-1671
-
-
Buiakova, O.I.1
Xu, J.2
Lutsenko, S.3
Zeitlin, S.4
Das, K.5
Das, S.6
-
10
-
-
59149094700
-
Atp7b-/- mice as a model for studies of Wilson's disease
-
S. Lutsenko Atp7b-/- mice as a model for studies of Wilson's disease Biochem Soc Trans 36 2008 1233 1238
-
(2008)
Biochem Soc Trans
, vol.36
, pp. 1233-1238
-
-
Lutsenko, S.1
-
11
-
-
0035999550
-
Chances and shortcomins of adenovirus-mediated ATP7B gene transfer in Wilson disease: Proof of principle demonstrated in a pilot study with LEC rats
-
D. Ha-Hao, U. Merle, C. Hofmann, H. Wesch, J. Doll, G. Auburger, and et al. Chances and shortcomins of adenovirus-mediated ATP7B gene transfer in Wilson disease: proof of principle demonstrated in a pilot study with LEC rats Z Gastroenterol 40 2002 209 216
-
(2002)
Z Gastroenterol
, vol.40
, pp. 209-216
-
-
Ha-Hao, D.1
Merle, U.2
Hofmann, C.3
Wesch, H.4
Doll, J.5
Auburger, G.6
-
12
-
-
33745712832
-
Lentiviral gene transfer ameliorates disease progression in Long-Evans cinnamon rats: An animal model for Wilson disease
-
U. Merle, J. Encke, S. Tuma, M. Volkmann, L. Naldini, and W. Stremmel Lentiviral gene transfer ameliorates disease progression in Long-Evans cinnamon rats: an animal model for Wilson disease Scand J Gastroenterol 41 2006 974 982
-
(2006)
Scand J Gastroenterol
, vol.41
, pp. 974-982
-
-
Merle, U.1
Encke, J.2
Tuma, S.3
Volkmann, M.4
Naldini, L.5
Stremmel, W.6
-
13
-
-
84911383748
-
Long-term safety and efficacy of factor IX gene therapy in hemophilia B
-
A.C. Nathwani, U.M. Reiss, E.G. Tuddenham, C. Rosales, P. Chowdary, J. McIntosh, and et al. Long-term safety and efficacy of factor IX gene therapy in hemophilia B N Engl J Med 371 2014 1994 2004
-
(2014)
N Engl J Med
, vol.371
, pp. 1994-2004
-
-
Nathwani, A.C.1
Reiss, U.M.2
Tuddenham, E.G.3
Rosales, C.4
Chowdary, P.5
McIntosh, J.6
-
14
-
-
0037366210
-
In vitro and in vivo comparative study of chimeric liver-specific promoters
-
M.G. Kramer, M. Barajas, N. Razquin, P. Berraondo, M. Rodrigo, C. Wu, and et al. In vitro and in vivo comparative study of chimeric liver-specific promoters Mol Ther 7 2003 375 385
-
(2003)
Mol Ther
, vol.7
, pp. 375-385
-
-
Kramer, M.G.1
Barajas, M.2
Razquin, N.3
Berraondo, P.4
Rodrigo, M.5
Wu, C.6
-
15
-
-
0024693747
-
Definition of an efficient synthetic poly(A) site
-
N. Levitt, D. Briggs, A. Gil, and N.J. Proudfoot Definition of an efficient synthetic poly(A) site Genes Dev 3 1989 1019 1025
-
(1989)
Genes Dev
, vol.3
, pp. 1019-1025
-
-
Levitt, N.1
Briggs, D.2
Gil, A.3
Proudfoot, N.J.4
-
16
-
-
73149122801
-
Reduced expression of ATP7B affected by Wilson disease-causing mutations is rescued by pharmacological folding chaperones 4-phenylbutyrate and curcumin
-
P.V. van den Berghe, J.M. Stapelbroek, E. Krieger, P. de Bie, S.F. van de Graaf, R.E. de Groot, and et al. Reduced expression of ATP7B affected by Wilson disease-causing mutations is rescued by pharmacological folding chaperones 4-phenylbutyrate and curcumin Hepatology 50 2009 1783 1795
-
(2009)
Hepatology
, vol.50
, pp. 1783-1795
-
-
Van Den Berghe, P.V.1
Stapelbroek, J.M.2
Krieger, E.3
De Bie, P.4
Van De Graaf, S.F.5
De Groot, R.E.6
-
17
-
-
66649118310
-
Therapeutic targeting of ATP7B in ovarian carcinoma
-
L.S. Mangala, V. Zuzel, R. Schmandt, E.S. Leshane, J.B. Halder, G.N. Armaiz-Pena, and et al. Therapeutic targeting of ATP7B in ovarian carcinoma Clin Cancer Res 15 2009 3770 3780
-
(2009)
Clin Cancer Res
, vol.15
, pp. 3770-3780
-
-
Mangala, L.S.1
Zuzel, V.2
Schmandt, R.3
Leshane, E.S.4
Halder, J.B.5
Armaiz-Pena, G.N.6
-
18
-
-
84880610768
-
Efficacy and safety of oral chelators in treatment of patients with Wilson disease
-
e1-e2
-
K.H. Weiss, F. Thurik, D.N. Gotthardt, M. Schäfer, U. Teufel, F. Wiegand, and et al. Efficacy and safety of oral chelators in treatment of patients with Wilson disease Clin Gastroenterol Hepatol 11 2013 1028 1035 e1-e2
-
(2013)
Clin Gastroenterol Hepatol
, vol.11
, pp. 1028-1035
-
-
Weiss, K.H.1
Thurik, F.2
Gotthardt, D.N.3
Schäfer, M.4
Teufel, U.5
Wiegand, F.6
-
19
-
-
84957711934
-
Zinc therapy for Wilson disease in children: A survey in French centers
-
[Epub ahead of print]
-
R. Santiago, F. Gottrand, D. Debray, L. Bridoux, A. Lachaux, A. Morali, and et al. Zinc therapy for Wilson disease in children: a survey in French centers J Pediatr Gastroenterol Nutr 2015 [Epub ahead of print]
-
(2015)
J Pediatr Gastroenterol Nutr
-
-
Santiago, R.1
Gottrand, F.2
Debray, D.3
Bridoux, L.4
Lachaux, A.5
Morali, A.6
-
20
-
-
84900020097
-
Liver transplantation for Wilson's disease
-
M.L. Schilsky Liver transplantation for Wilson's disease Ann N Y Acad Sci 1315 2014 45 49
-
(2014)
Ann N y Acad Sci
, vol.1315
, pp. 45-49
-
-
Schilsky, M.L.1
-
21
-
-
0029851752
-
Intrahepatic transplantation of normal hepatocytes prevents Wilson's disease in Long-Evans cinnamon rats
-
Y. Yoshida, Y. Tokusashi, G.H. Lee, and K. Ogawa Intrahepatic transplantation of normal hepatocytes prevents Wilson's disease in Long-Evans cinnamon rats Gastroenterology 111 1996 1654 1660
-
(1996)
Gastroenterology
, vol.111
, pp. 1654-1660
-
-
Yoshida, Y.1
Tokusashi, Y.2
Lee, G.H.3
Ogawa, K.4
-
22
-
-
0034981021
-
Correction of liver disease following transplantation of normal rat hepatocytes into Long-Evans Cinnamon rats modeling Wilson's disease
-
A.N. Irani, H. Malhi, S. Slehria, G.R. Gorla, I. Volenberg, M.L. Schilsky, and et al. Correction of liver disease following transplantation of normal rat hepatocytes into Long-Evans Cinnamon rats modeling Wilson's disease Mol Ther 3 2001 302 309
-
(2001)
Mol Ther
, vol.3
, pp. 302-309
-
-
Irani, A.N.1
Malhi, H.2
Slehria, S.3
Gorla, G.R.4
Volenberg, I.5
Schilsky, M.L.6
-
23
-
-
40549138765
-
Processing of recombinant AAV genomes occurs in specific nuclear structures that overlap with foci of DNA-damage-response proteins
-
T. Cervelli, J.A. Palacios, L. Zentilin, M. Mano, R.A. Schwartz, M.D. Weitzman, and et al. Processing of recombinant AAV genomes occurs in specific nuclear structures that overlap with foci of DNA-damage-response proteins J Cell Sci 121 2008 349 357
-
(2008)
J Cell Sci
, vol.121
, pp. 349-357
-
-
Cervelli, T.1
Palacios, J.A.2
Zentilin, L.3
Mano, M.4
Schwartz, R.A.5
Weitzman, M.D.6
-
24
-
-
0033766166
-
Chronic ethanol increases adeno-associated viral transgene expression in rat liver via oxidant and NFkappaB-dependent mechanisms
-
M.D. Wheeler, H. Kono, I. Rusyn, G.E. Arteel, D. McCarty, R.J. Samulski, and et al. Chronic ethanol increases adeno-associated viral transgene expression in rat liver via oxidant and NFkappaB-dependent mechanisms Hepatology 32 2000 1050 1059
-
(2000)
Hepatology
, vol.32
, pp. 1050-1059
-
-
Wheeler, M.D.1
Kono, H.2
Rusyn, I.3
Arteel, G.E.4
McCarty, D.5
Samulski, R.J.6
-
25
-
-
79958061881
-
AAV exploits subcellular stress associated with inflammation, endoplasmic reticulum expansion, and misfolded proteins in models of cystic fibrosis
-
J.S. Johnson, M. Gentzsch, L. Zhang, C.M. Ribeiro, B. Kantor, T. Kafri, and et al. AAV exploits subcellular stress associated with inflammation, endoplasmic reticulum expansion, and misfolded proteins in models of cystic fibrosis PLoS Pathog 7 2011 e1002053
-
(2011)
PLoS Pathog
, vol.7
, pp. e1002053
-
-
Johnson, J.S.1
Gentzsch, M.2
Zhang, L.3
Ribeiro, C.M.4
Kantor, B.5
Kafri, T.6
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