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- W1977972524 abstract "Wilson’s disease (WD) is a severe disorder of copper misbalance, which manifests with a wide spectrum of liver pathology and/or neurologic and psychiatric symptoms. WD is caused by mutations in a gene encoding a copper-transporting ATPase ATP7B and is accompanied by accumulation of copper in tissues, especially in the liver. Copper-chelation therapy is available for treatment of WD symptoms and is often successful, however, significant challenges remain with respect to timely diagnostics and treatment of the disease. The lack of genotype-phenotype correlation remains unexplained, the causes of fulminant liver failure are not known, and the treatment of neurologic symptoms is only partially successful, underscoring the need for better understanding of WD mechanisms and factors that influence disease manifestations. Recent gene and protein profiling studies in animal models of WD began to uncover cellular processes that are primarily affected by copper accumulation in the liver. The results of such studies, summarized in this review, revealed new molecular players and pathways (cell cycle and cholesterol metabolism, mRNA splicing and nuclear receptor signaling) linked to copper misbalance. A systems biology approach promises to generate a comprehensive view of WD onset and progression, thus helping with a more fine-tune treatment and monitoring of the disorder." @default.
- W1977972524 created "2016-06-24" @default.
- W1977972524 creator A5046814999 @default.
- W1977972524 creator A5075320684 @default.
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- W1977972524 date "2011-03-05" @default.
- W1977972524 modified "2023-10-10" @default.
- W1977972524 title "Systems biology approach to Wilson’s disease" @default.
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- W1977972524 cites W1542765837 @default.
- W1977972524 cites W1964908479 @default.
- W1977972524 cites W1965367852 @default.
- W1977972524 cites W1965415623 @default.
- W1977972524 cites W1966296560 @default.
- W1977972524 cites W1969997274 @default.
- W1977972524 cites W1972044040 @default.
- W1977972524 cites W1973178590 @default.
- W1977972524 cites W1976688665 @default.
- W1977972524 cites W1978415684 @default.
- W1977972524 cites W1979937961 @default.
- W1977972524 cites W1980441473 @default.
- W1977972524 cites W1983723576 @default.
- W1977972524 cites W1984319030 @default.
- W1977972524 cites W1986541184 @default.
- W1977972524 cites W1993864942 @default.
- W1977972524 cites W1994758176 @default.
- W1977972524 cites W1996211742 @default.
- W1977972524 cites W1999577642 @default.
- W1977972524 cites W2002023508 @default.
- W1977972524 cites W2004576317 @default.
- W1977972524 cites W2007131731 @default.
- W1977972524 cites W2007728277 @default.
- W1977972524 cites W2017117389 @default.
- W1977972524 cites W2020987797 @default.
- W1977972524 cites W2023051967 @default.
- W1977972524 cites W2024544489 @default.
- W1977972524 cites W2026564074 @default.
- W1977972524 cites W2027910199 @default.
- W1977972524 cites W2043161246 @default.
- W1977972524 cites W2044468018 @default.
- W1977972524 cites W2054081976 @default.
- W1977972524 cites W2054687549 @default.
- W1977972524 cites W2055851605 @default.
- W1977972524 cites W2057578633 @default.
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- W1977972524 cites W2058835240 @default.
- W1977972524 cites W2068551564 @default.
- W1977972524 cites W2084248691 @default.
- W1977972524 cites W2088042071 @default.
- W1977972524 cites W2089738642 @default.
- W1977972524 cites W2095874720 @default.
- W1977972524 cites W2097430018 @default.
- W1977972524 cites W2097951502 @default.
- W1977972524 cites W2103180882 @default.
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- W1977972524 cites W2114090652 @default.
- W1977972524 cites W2114518943 @default.
- W1977972524 cites W2117901133 @default.
- W1977972524 cites W2118480909 @default.
- W1977972524 cites W2129876249 @default.
- W1977972524 cites W2130074105 @default.
- W1977972524 cites W2132722165 @default.
- W1977972524 cites W2133282596 @default.
- W1977972524 cites W2134359770 @default.
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- W1977972524 cites W2136476112 @default.
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- W1977972524 cites W2147286697 @default.
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- W1977972524 cites W2148487430 @default.
- W1977972524 cites W2149082710 @default.
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- W1977972524 cites W2165101124 @default.
- W1977972524 cites W2166240320 @default.
- W1977972524 cites W2168114154 @default.
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- W1977972524 doi "https://doi.org/10.1007/s10534-011-9430-9" @default.
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