ArticleJournal of molecular biology2011
Elevated copper remodels hepatic RNA processing machinery in the mouse model of Wilson's disease.
Article in Journal of molecular biology, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 38 citations in OpenAlex.
- Tackling cuproptosis: from metabolic rewiring to therapeutic exploitation in cancer.Cellular & molecular immunology · 2026Review
- Mammalian copper homeostasis: physiological roles and molecular mechanisms.Physiological reviews · 2025Review
- Identification of lncRNA-miRNA-mRNA Networks in the Lenticular Nucleus Region of the Brain Contributes to Hepatolenticular Degeneration Pathogenesis and Therapy.Molecular neurobiology · 2024Article
- Wilson Disease: Update on Pathophysiology and Treatment.Frontiers in cell and developmental biology · 2022Review
- Heterogeneous nuclear ribonucleoprotein hnRNPA2/B1 regulates the abundance of the copper-transporter ATP7A in an isoform-dependent manner.Frontiers in molecular biosciences · 2022Article
- High-dose copper activates p53-independent apoptosis through the induction of nucleolar stress in human cell lines.Apoptosis : an international journal on programmed cell death · 2021Article
- Dynamic and cell-specific transport networks for intracellular copper ions.Journal of cell science · 2021Review
- Article
- Copper interferes with selenoprotein synthesis and activity.Redox biology · 2020Article
- The histone H3-H4 tetramer is a copper reductase enzyme.Science (New York, N.Y.) · 2020Article
- Animal models of Wilson disease.Journal of neurochemistry · 2018Review
- Targeted inactivation of copper transporter Atp7b in hepatocytes causes liver steatosis and obesity in mice.American journal of physiology. Gastrointestinal and liver physiology · 2017Article
- The genetics of Wilson disease.Handbook of clinical neurology · 2017Review
- Copper transporters and chaperones: Their function on angiogenesis and cellular signalling.Journal of biosciences · 2016Review
- The Activity of Menkes Disease Protein ATP7A Is Essential for Redox Balance in Mitochondria.The Journal of biological chemistry · 2016Article
- Annexin A5 is the Most Abundant Membrane-Associated Protein in Stereocilia but is Dispensable for Hair-Bundle Development and Function.Scientific reports · 2016Article
- P-coumaric acid regulates exon 12 splicing of the ATP7B gene by modulating hnRNP A1 protein expressions.BioMedicine · 2015Article
- Modifying factors and phenotypic diversity in Wilson's disease.Annals of the New York Academy of Sciences · 2014Review
- Wilson's disease: changes in methionine metabolism and inflammation affect global DNA methylation in early liver disease.Hepatology (Baltimore, Md.) · 2013Article
- Selenium. Role of the essential metalloid in health.Metal ions in life sciences · 2013Review
Corrections and comments
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Authors and funding
5 authors at 3 institutions in 1 country.
Funding
Abstract
Copper is essential to mammalian physiology, and its homeostasis is tightly regulated. In humans, genetic defects in copper excretion result in copper overload and Wilson's disease (WD). Previous studies on the mouse model for WD (Atp7b(-)(/-)) revealed copper accumulation in hepatic nuclei and specific changes in mRNA profile prior to the onset of pathology. To find a molecular link between nuclear copper elevation and changes in hepatic transcriptome, we utilized quantitative ionomic and proteomic approaches. X-ray fluorescence and inductively coupled plasma mass spectrometry analysis indicate that copper in the Atp7b(-/-) nucleus, while highly elevated, does not markedly alter nuclear ion content. Widespread protein oxidation is also not observed, although the glutathione reductase SelH is upregulated, likely to maintain redox balance. We further demonstrate that accumulating copper affects the abundance and/or modification of a distinct subset of nuclear proteins. These proteins populate pathways that are most significantly associated with RNA processing. An alteration in splicing pattern was observed for hnRNP A2/B1, itself the RNA shuttling factor and spliceosome component. Analysis of hnRNP A2/B1 mRNA and protein revealed an increased retention of exon 2 and a selective 2-fold upregulation of a corresponding protein splice variant. Mass spectrometry measurements suggest that the nucleocytoplasmic distribution of RNA binding proteins, including hnRNP A2/B1, is altered in the Atp7b(-/-) liver. We conclude that remodeling of the RNA processing machinery is an important component of cell response to elevated copper that may guide pathology development in the early stages of WD.
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Registered trials
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