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1 iency (Menkes syndrome) and copper overload (Wilson disease).
2  mutants and, hence, to counter the onset of Wilson disease.
3 netic diseases, including Menkes disease and Wilson disease.
4 is due to a gene different from the gene for Wilson disease.
5 s property has pharmacologic applications in Wilson disease.
6 ellular copper transport, Menkes disease and Wilson disease.
7 he genes responsible for Menkes syndrome and Wilson disease.
8 otentially fatal hepatoneurological disorder Wilson disease.
9 count for gastrointestinal manifestations of Wilson disease.
10 or the development of novel therapeutics for Wilson disease.
11 e (PEN) are used to treat copper overload in Wilson disease.
12 jor site of Cu accumulation in patients with Wilson disease.
13 argeted for therapeutic approaches to combat Wilson disease.
14 and, thereby, to hepatic copper toxicosis in Wilson disease.
15 principle will advance molecular imaging for Wilson disease.
16 s agouti (LEA) rats versus LEC rats modeling Wilson disease.
17 per transporter Atp7b, which is deficient in Wilson disease.
18 e function of its product and development of Wilson disease.
19 the genetic diagnosis of hemochromatosis and Wilson disease.
20                   Mutations in ATP7B lead to Wilson disease.
21 alysis for Atp7b-/- mice, an animal model of Wilson disease.
22 cess it causes the severe metabolic disorder Wilson disease.
23 e copper-toxicity-related characteristics of Wilson disease.
24 ly related copper ATPase, ATP7B, affected in Wilson disease.
25          Mutations in the ATP7B gene lead to Wilson disease, a copper toxicity disorder characterized
26 tial yet toxic metal and its overload causes Wilson disease, a disorder due to mutations in copper tr
27 in labile copper levels in a murine model of Wilson disease, a genetic disorder that is characterized
28                                    The first Wilson Disease Aarhus Symposium included a workshop on r
29 o synapses.SIGNIFICANCE STATEMENT Menkes and Wilson disease affect copper homeostasis and characteris
30 osphorylation, and mutations associated with Wilson disease alter the steady-state distribution of AT
31 nected to severe diseases such as Menkes and Wilson diseases, Alzheimer's disease, prion disorders, a
32 romatosis and other iron overload disorders, Wilson disease and alpha one antitrypsin deficiency.
33  the current clinical and research status of Wilson disease and canine copper toxicosis.
34                                              Wilson disease and genetic hemochromatosis involve defec
35 factors that modify phenotypic expression of Wilson disease and genetic hemochromatosis.
36 opper and iron improved our understanding of Wilson disease and genetic hemochromatosis.
37 s Cinnamon rat identifies an animal model of Wilson disease and will permit experimental analysis of
38 ar to the copper pumps related to Menkes and Wilson diseases and provides a useful prokaryotic model
39 tations in ATP7B cause the potentially fatal Wilson disease, and changes in ATP7B expression are obse
40 elenium levels are decreased in tissues of a Wilson disease animal model, especially in the liver, ma
41                           Menkes disease and Wilson disease are human disorders of copper transport c
42                                   Menkes and Wilson diseases are associated with retinal degeneration
43         This is the case for both Menkes and Wilson disease, arising from mutations in ATP7A and ATP7
44                                   ATP7B, the Wilson disease-associated Cu(I)-transporter, and ZntA fr
45  were uniquely effective for cell therapy in Wilson disease because of the nature of preexisting hepa
46 -CLD if diagnosed with autoimmune hepatitis, Wilson disease, Budd-Chiari syndrome, hepatitis B virus
47 ne complex would be effective for diagnosing Wilson disease by molecular imaging and tested this hypo
48                                              Wilson disease can have clinical and laboratory features
49 deficiency and toxicity disorders Menkes and Wilson diseases caused by mutations in the p-type Cu-ATP
50 the degradation of ATP7B containing the same Wilson disease-causing C-terminal mutations via differen
51 he hepatocytes and rescues the most frequent Wilson-disease-causing ATP7B mutant to the appropriate f
52 copper-induced trafficking of the Menkes and Wilson disease copper ATPases is associated with the pho
53 dent interaction of isolated N-MBDs from the Wilson disease Cu-ATPase with the ATP binding cytoplasmi
54        Cu chelators used in the treatment of Wilson disease decreased tumour growth of human or murin
55            Variants in ATP7B associated with Wilson disease disrupt the protein's transport activity,
56 ted samples from 92 real-world patients with Wilson disease during a 2-year period.
57 fied, is produced as a truncated form of the Wilson disease gene (Atp7b) product.
58 revisiae deficient in CCC2, the yeast Menkes/Wilson disease gene homologue.
59                           The cloning of the Wilson disease gene opened up the possibility that a dir
60 he yeast homolog of MNK and WND (ATP7B), the Wilson disease gene product.
61 e that are homozygous mutants (null) for the Wilson disease gene.
62 7A and ATP7B, the products of the Menkes and Wilson disease genes, respectively, catalyze an ATP-depe
63  our understanding of the pathophysiology of Wilson disease, genetic hemochromatosis and alpha-1 anti
64                     Three selected disorders-Wilson disease, genetic hemochromatosis and other heredi
65 e following section focuses on the disorders Wilson disease hemochromatosis and alpha-one antitrypsin
66                        Four selected topics, Wilson disease, hemochromatosis and iron overload disord
67 tudy Alagille syndrome, fatty liver disease, Wilson disease, hepatitis B viral infection, and cystic
68 rm of cirrhotic liver disease that resembles Wilson disease in humans and the 'toxic milk' phenotype
69 gradation constitutes the molecular basis of Wilson disease in patients harboring the H1069Q mutation
70                           BACKGROUND & AIMS: Wilson disease is a disorder of copper (Cu) misbalance c
71                                              Wilson disease is a disorder of copper metabolism charac
72                                              Wilson disease is a severe disorder of copper metabolism
73                                              Wilson disease is a severe genetic disorder associated w
74                                              Wilson disease is an autosomal recessive disorder of hep
75 n of saccades to presented stimulus, whereas Wilson disease is associated with saccadic pursuits, inc
76       One of the main clinical challenges in Wilson disease is for clinicians to recognize the possib
77 overies for both iron overload disorders and Wilson disease is our increasing understanding that the
78 o normal intestinal copper homeostasis or to Wilson disease manifestations.
79   Patients with neurologic disorders such as Wilson disease may first present with decreased vision a
80 ncements in diagnosis of hemochromatosis and Wilson disease may lead to earlier diagnosis and treatme
81 unts for the disruptive effects of >30 known Wilson disease mutations.
82  with ALF-CLD (autoimmune hepatitis, n = 14; Wilson disease, n = 6; inborn errors of metabolism, n =
83 quired for copper homeostasis and related to Wilson disease of humans.
84 gnosis and management of hemochromatosis and Wilson disease over the past 18 months.
85 n a copper-transporting P1B-type ATPase of a Wilson disease patient, the PCA1 allele found in laborat
86                             These mice, like Wilson disease patients, have intracellular copper accum
87 Perturbation of the human copper-transporter Wilson disease protein (ATP7B) causes intracellular copp
88                                              Wilson disease protein (ATP7B) is a copper-transporting
89 x1 interacts with Menkes disease protein and Wilson disease protein (WD) and functions in copper effl
90                                          The Wilson disease protein (WND) is a transport ATPase invol
91 opper transport by the P(1)-ATPase ATP7B, or Wilson disease protein (WNDP),1 is essential for human m
92    ATP7A is a faster copper transporter than Wilson disease protein as evidenced by faster rates of c
93                                          The Wilson disease protein ATP7B exhibits copper-dependent t
94                 Here we demonstrate that the Wilson disease protein directly interacts with the human
95                                          The Wilson disease protein is a copper transporting ATPase s
96 a coli K+-transporting Kdp-ATPase and to the Wilson disease protein N-domain indicate that the five c
97                       Inactivation of ATP7B (Wilson disease protein) by gene knock-out induces a stri
98 an ATP7A (Menkes disease protein) and ATP7B (Wilson disease protein), as well as yeast Atx1, a cytoso
99 st common disease mutation in the homologous Wilson disease protein.
100 t is closely related to the human Menkes and Wilson disease proteins was cloned.
101 (MBDs) of the two P-type ATPases (Menkes and Wilson disease proteins), the destination for copper bou
102 type ATPases Atp7a and Atp7b (the Menkes and Wilson disease proteins, respectively), which are respon
103 rotein and related domains in the Menkes and Wilson disease proteins.
104 ntified, but the molecular events leading to Wilson disease remain poorly understood.
105 ng suitable copper parameters for monitoring Wilson disease remains a topic of ongoing discussion.
106 7B, genes associated with Menkes disease and Wilson disease, respectively, revealed that expression o
107 ses ATP7A and ATP7B cause Menkes disease and Wilson disease, respectively.
108 opper imbalance syndromes Menkes disease and Wilson disease, respectively.
109 rodegenerative disorders, Menkes disease and Wilson disease, respectively.
110  homeostasis, and mutations cause Menkes and Wilson diseases, respectively.
111 cy and copper toxicity disorders, Menkes and Wilson diseases, respectively.
112 rug hepatitis, fatty liver, hemochromatosis, Wilson disease, several biliary tract disorders, and pat
113         His initial workup was suggestive of Wilson disease-subsequent ATP7B gene was negative.
114 yzed 28 variants of ATP7B from patients with Wilson disease that affected different functional domain
115 ogous to one of human copper ATPase ATP7B in Wilson disease) that inhibits ATPase activity.
116                                          For Wilson disease, there is new data regarding the structur
117 the data were obtained in an animal model of Wilson' disease, these biochemical parameters likely ref
118     BeWo cells may also express the gene for Wilson disease, thus linking Menkes and Wilson proteins
119               Normal reference intervals for Wilson disease-treated patients should be redefined, as
120                                              Wilson disease (WD) and Menkes disease (MNK) are inherit
121                 The pathogenic agent of both Wilson disease (WD) and non-Indian childhood cirrhosis (
122 ngs at brain MRI in patients with neurologic Wilson disease (WD) are characterized by signal intensit
123               The clinical manifestations of Wilson disease (WD) are related to copper accumulation i
124                  Conclusion Individuals with Wilson disease (WD) had widespread brain atrophy most pr
125                     Clinical presentation of Wilson disease (WD) includes hepatic and neurologic mani
126                                              Wilson disease (WD) is a hepatoneurological disorder cau
127                                              Wilson disease (WD) is a metabolic disorder caused by in
128                                              Wilson disease (WD) is a monogenic autosomal-recessive d
129                                              Wilson disease (WD) is a monogenic disorder caused by mu
130                                              Wilson disease (WD) is a severe hepato-neurologic disord
131                                              Wilson disease (WD) is an autosomal recessive disorder c
132                                              Wilson disease (WD) is an autosomal recessive disorder t
133                                              Wilson disease (WD) is an autosomal-recessive disorder c
134                                              Wilson disease (WD) is an inherited disorder of copper m
135                                              Wilson disease (WD) is an inherited disorder of hepatic
136      The severe liver pathology of untreated Wilson disease (WD) is associated with massive copper ov
137                                              Wilson disease (WD) is caused by inactivation of the cop
138             Acute liver failure (ALF) due to Wilson disease (WD) is invariably fatal without emergenc
139 per metabolism correction in 6-week-old male Wilson disease (WD) mice.
140 tox1 delivers Cu to metal-binding domains of Wilson disease (WD) protein for insertion into cuproenzy
141                                              Wilson Disease (WD) usually presents in the first decade
142                          Novel therapies for Wilson disease (WD) will require appropriate biomarkers
143                          Hemochromatosis and Wilson disease (WD), characterized by the excess hepatic
144                                           In Wilson disease (WD), functional loss of ATPase copper-tr
145 plice variant of the ATP7B gene disrupted in Wilson disease (WD).
146  accumulation and severe pathology, known as Wilson disease (WD).
147  accumulation and severe pathology, known as Wilson disease (WD).
148 cacy of pro-oxidant hepatic perturbations in Wilson disease, we studied Long-Evans Cinnamon (LEC) rat
149 r clinicians to recognize the possibility of Wilson disease when young patients present with liver di
150 nce of genotype and phenotype correlation in Wilson disease, which can be diagnosed by genetic sequen
151 ideline has been developed for children with Wilson disease with mild liver disease that increases th
152                                              Wilson disease (WND) is caused by inactivation of the co
153 , using a 170-kb clone containing the entire Wilson disease (WND) locus as a model.

 
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