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1 ncept of selective neuronal vulnerability in Wilson's disease.
2 ns in the copper transporter Atp7b result in Wilson's disease.
3  with clinically and biochemically confirmed Wilson's disease.
4 strong evidence for monogenic inheritance of Wilson's disease.
5  The Atp7b (-/-) mouse develops hallmarks of Wilson's disease.
6 rypsin deficiency, autoimmune hepatitis, and Wilson's disease.
7  of this lesion in tissues of a rat model of Wilson's disease.
8 or drugs that prevent copper accumulation in Wilson's disease.
9 progression of neurological diseases such as Wilson's disease.
10  constitute potential therapeutic agents for Wilson's disease.
11 in WNDP lead to a severe metabolic disorder, Wilson's disease.
12 and C virus infections, hemochromatosis, and Wilson's disease.
13 ease progression and therapeutic response in Wilson's disease.
14 Long Evans Cinnamon rats, an animal model of Wilson's disease.
15 could noninvasively assess liver function in Wilson's disease.
16 d to a severe multisystem disorder, known as Wilson's disease.
17 , 21 OLTs were performed in 17 patients with Wilson's disease.
18  initial or early treatment of patients with Wilson's disease.
19 ew our experience with OLT for patients with Wilson's disease.
20  directly contribute to neurodegeneration in Wilson's disease.
21                   Mutations in ATP7B lead to Wilson's disease, a severe disorder with neurological an
22 rom 40 prospectively recruited patients with Wilson's disease (age range 16-68).
23                                              Wilson's disease, an autosomal disorder associated with
24 ns of copper in urine samples from mice with Wilson's disease and also tracing exogenously added copp
25                                         Both Wilson's disease and genetic hemochromatosis involve def
26 er toxicity contributes to neuronal death in Wilson's disease and has been speculatively linked to th
27  in the pathogenesis of Friedreich's ataxia, Wilson's disease and hereditary spastic paraplegia.
28 ved in liver carcinogenesis in patients with Wilson's disease and in other human cancers.
29 ossible mechanism of action in arthritis and Wilson's disease and may also underlie complications ass
30 uced by intracellular copper accumulation in Wilson's disease and other copper toxicosis disorders an
31 rrhosis, biliary cirrhosis, hemochromatosis, Wilson's disease) and ICC (biliary cirrhosis, cholangiti
32 um (calcifications, Fahr's disease), copper (Wilson's disease) and manganese (hepatic encephalopathy,
33 . anaemia, haemochromatosis, Menkes disease, Wilson's disease), and neurodegenerative diseases (i.e.
34 the pathogenesis of genetic hemochromatosis, Wilson's disease, and alpha1-antitrypsin deficiency grow
35 Cu transport rates during copper deficiency, Wilson's disease, and other copper toxicosis syndromes.
36 ailable to confirm the clinical diagnosis of Wilson's disease, and results from biochemical and genet
37 siological clusters isolated from TM-treated Wilson's disease animal models.
38  which we were able to successfully identify Wilson's disease animals from healthy control mice in ea
39 lassemia) at a rate of about 1 in 28, ATP7B (Wilson's disease) at a rate of about 1 in 40, PAH (Pheny
40                       Clinical management of Wilson's disease, autoimmune liver disease, and chronic
41 ping targeted molecular and gene therapy for Wilson's disease, before discussing future directions fo
42                                              Wilson's disease can present with hepatic and neurologic
43                                   Therapy of Wilson's disease continues to evolve.
44 transplantation for hepatic complications of Wilson's disease cures and corrects the underlying metab
45               We report the first cases with Wilson's disease due to segmental uniparental isodisomy
46 opper metabolism, namely Menkes syndrome and Wilson's disease, encode P-type ATPases that are more si
47 es of copper metabolism, Menkes syndrome and Wilson's disease, encode P-type ATPases that are more si
48  pathogenesis, biomarkers and treatments for Wilson's disease from the neurological perspective, with
49 ies have failed to identify mutations in the Wilson's disease gene ATP7B in a significant number of c
50                         The discovery of the Wilson's disease gene has opened up a new molecular diag
51 le patients (hepatitis B; hepatitis B and D; Wilson's disease; hepatitis B, D, and C; and 3 with hepa
52      We determined the genetic prevalence of Wilson's disease in the United Kingdom by sequencing the
53 hree ATP7B mutations and three families with Wilson's disease in two consecutive generations.
54                Eleven patients had fulminant Wilson's disease; in six patients the presentation was c
55                                              Wilson's disease invariably results in severe disability
56                                              Wilson's disease is a genetic disorder in which copper a
57                                              Wilson's disease is an autosomal-recessive disorder of c
58                                              Wilson's disease is an autosomal-recessive disorder of c
59                                              Wilson's disease is an inherited disorder of copper meta
60                           Early diagnosis of Wilson's disease is crucial to ensure that patients can
61 rogressive hepatolenticular degeneration, or Wilson's disease, is a genetic disorder of copper metabo
62 vans Cinnamon (LEC) rat, an animal model for Wilson's disease, is an inbred mutant strain, which beca
63 etic defects of copper distribution, such as Wilson's disease, lead to severe pathologies, including
64  the number of clinically diagnosed cases of Wilson's disease may be due to both reduced penetrance o
65  and genetic prevalence studies suggest that Wilson's disease might be much more common than previous
66 y wild-type mice as well as elevated Cu in a Wilson's disease model and in a liver metastasis model.
67 efects in copper metabolism cause Menkes and Wilson's disease, myeloneuropathy, and cardiovascular di
68                                 Furthermore, Wilson's disease needs to be differentiated from other c
69 er than the typically reported prevalence of Wilson's disease of 1:30 000 (P = 0.00093).
70       It is universally agreed that pregnant Wilson's disease patients should remain on anticopper th
71 utations in genes other than ATP7B causing a Wilson's disease phenotype is therefore very low.
72 ies were reduced in the Atp7b mouse model of Wilson's disease prior to liver damage, and were partial
73             The association of ClC-4 and the Wilson's disease protein (ATP7B) was determined by co-im
74  copper chaperone Hah1 delivers Cu(+) to the Wilson's Disease Protein (WDP) via weak and dynamic prot
75  the copper-transporting ATPase ATP7B or the Wilson's disease protein (WNDP) belongs to the large fam
76                                          The Wilson's disease protein (WNDP) is a copper-transporting
77                                              Wilson's disease protein (WNDP) is a copper-transporting
78       The mutations in the ATPase ATP7B, the Wilson's disease protein (WNDP), lead to intracellular a
79 phorylation of a key copper transporter, the Wilson's disease protein (WNDP).
80                             Cu-ATPase ATP7B (Wilson's disease protein) transports copper into the tra
81  intracellular mislocalization of ATP7B (the Wilson's disease protein, WNDP).
82  gene encoding a copper-transporting ATPase (Wilson's disease protein, WNDP).
83 e, present in 45% of patients with fulminant Wilson's disease, resolved post-OLT with supportive care
84                                              Wilson's disease results from mutations in the P-type Cu
85 taxias, myorhythmia, isolated tongue tremor, Wilson's disease, slow orthostatic tremor, peripheral tr
86              For genetic hemochromatosis and Wilson's disease, studies focused on the function of the
87 tion or share biochemical abnormalities with Wilson's disease, such as reduced serum ceruloplasmin co
88 f these observations for conditions, such as Wilson's disease, that can involve raised Cu(2+) levels
89 rg and Sternlieb estimated the prevalence of Wilson's disease to be 1:30,000 based on the limited ava
90 3207C>A mutation in the ATP7B gene linked to Wilson's disease, upregulating the PDX1 gene expression
91 or all relevant studies on the prevalence of Wilson's disease was conducted.
92 f non-HFE hemochromatosis were published and Wilson's disease was described in individuals of 60 year
93               The copper metabolism disorder Wilson's disease was first defined in 1912.
94                                     However, Wilson's disease was ruled out with normal serum cerulop
95                                              Wilson's disease (WD) is a genetic disease with systemic
96                                              Wilson's disease (WD) is a rare hereditary disorder due
97                                              Wilson's disease (WD) is an autosomal recessive disorder
98                                              Wilson's disease (WD) is an autosomal-recessive disorder
99                                              Wilson's disease (WD) is caused by mutations in a P-type
100 ating methylation status and liver injury in Wilson's disease (WD) through the inhibition of S-adenos
101                                           In Wilson's disease (WD), this function is disrupted due to
102 pper uptake, an effect utilized for treating Wilson's disease (WD).
103 pper excretion result in copper overload and Wilson's disease (WD).
104  been reported as mutations in patients with Wilson's disease were included in the analysis (Class 2
105                       Neurologic features of Wilson's disease were not prominent preoperatively and d
106 l overdose, 2 idiosyncratic drug reaction, 3 Wilson's disease) were available.
107 sorders of Cu metabolism, Menkes disease and Wilson's disease, which paved the way for novel approach
108 on (OLT) can be lifesaving for patients with Wilson's disease who present with fulminant liver failur
109      We enrolled patients (>/=18 years) with Wilson's disease who were untreated or had received no m
110 g-Evans Cinnamon (LEC) rats, which reproduce Wilson's disease with copper toxicosis, and their normal
111  be a promising new therapeutic approach for Wilson's disease, with a unique mode of action.
112                                              Wilson's disease (WND) is an inherited disorder of coppe

 
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