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TranscriptomicsTranscription profiling of mouse model of Wilson disease reveals specific changes of liver transcriptome in the early stages of copper accumulation
Wilson disease (WD) is a severe metabolic disorder caused by genetic inactivation of copper-transporting ATPase ATP7B. In WD, copper accumulates in several tissues, particularly in the liver, inducing marked time-dependent pathological changes. To identify initial events in the copper-dependent de...
ORGANISM(S): Mus musculus 
2008-06-13 | E-GEOD-5348 | biostudies-arrayexpress
Proteomicsα-Lipoic acid ameliorates consequences of copper overload by upregulating selenoproteins and decreasing redox misbalance
α-Lipoic acid (LA) is an essential cofactor for mitochondrial dehydrogenases and is required for cell growth, metabolic fuels production, and antioxidant defense. In vitro, LA binds copper (Cu) with high affinity, and as an endogenous membrane permeable metabolite could be advantageous in mitigating...
ORGANISM(S): Mus musculus (Mouse) 
2024-05-23 | PXD045233 | Pride
Α-lipoic acid Selenoprotein Copper Wilson disease Oxidative stress 
UnknownThe 100 top-cited articles in the field of Wilson's disease from 1990 to 2022: A bibliometric study.
Not available
S-EPMC10336511 | biostudies-literature
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