ArticleScientific reports2021
Systemic deletion of Atp7b modifies the hepatocytes' response to copper overload in the mouse models of Wilson disease.
Article in Scientific reports, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 51 citations in OpenAlex.
- Neutrophil extracellular traps delivering lactylated S100a9 aggravate neuronal cuproptosis by regulating Ttr/Commd1/Atp7b axis after traumatic brain injury.Redox biology · 2026Article
- Metallothionein and neurodegenerative diseases.Neural regeneration research · 2026Article
- Cuproptosis in Sepsis: Cell Type-Specific Mechanisms and Clinical Prospects.Drug design, development and therapy · 2026Review
- Comparative transcriptome analysis reveals genes associated with variation in liver copper concentration in Polish Merino sheep.Scientific reports · 2025Article
- Glutathione Attenuates Copper Levels and Alleviates Hepatic Injury in TX Mice.Biological trace element research · 2025Article
- Changes in the FXR-cistrome and alterations in bile acid physiology in Wilson disease.Hepatology communications · 2025Article
- Hepatic microtubule destabilization facilitates liver fibrosis in the mouse model of Wilson disease.Journal of molecular medicine (Berlin, Germany) · 2025Article
- Prion protein promotes copper toxicity in Wilson disease.Nature communications · 2025Article
- Mechanisms of Copper-Induced Autophagy and Links with Human Diseases.Pharmaceuticals (Basel, Switzerland) · 2025Review
- Mammalian copper homeostasis: physiological roles and molecular mechanisms.Physiological reviews · 2025Review
- Article
- Triptolide-induced cuproptosis is a novel antitumor strategy for the treatment of cervical cancer.Cellular & molecular biology letters · 2024Article
- Hepatic oxylipin profiles in mouse models of Wilson disease: New insights into early hepatic manifestations.Biochimica et biophysica acta. Molecular and cell biology of lipids · 2024Article
- Dysfunction in atox-1 and ceruloplasmin alters labile Cu levels and consequently Cu homeostasis inFrontiers in molecular biosciences · 2024Article
- Article
- Therapeutic implications of impaired nuclear receptor function and dysregulated metabolism in Wilson's disease.Pharmacology & therapeutics · 2023Review
- α-lipoic acid ameliorates consequences of copper overload by up-regulating selenoproteins and decreasing redox misbalance.Proceedings of the National Academy of Sciences of the United States of America · 2023Article
- Atp7b-dependent choroid plexus dysfunction causes transient copper deficit and metabolic changes in the developing mouse brain.PLoS genetics · 2023Article
- Mitochondrial copper in human genetic disorders.Trends in endocrinology and metabolism: TEM · 2023Review
- Copper induces cell death by targeting lipoylated TCA cycle proteins.Science (New York, N.Y.) · 2022Article
Corrections and comments
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Authors and funding
11 authors at 1 institution in 1 country.
Funding
Abstract
Wilson disease (WD) is caused by inactivation of the copper transporter Atp7b and copper overload in tissues. Mice with Atp7b deleted either globally (systemic inactivation) or only in hepatocyte recapitulate various aspects of human disease. However, their phenotypes vary, and neither the common response to copper overload nor factors contributing to variability are well defined. Using metabolic, histologic, and proteome analyses in three Atp7b-deficient mouse strains, we show that global inactivation of Atp7b enhances and specifically modifies the hepatocyte response to Cu overload. The loss of Atp7b only in hepatocytes dysregulates lipid and nucleic acid metabolisms and increases the abundance of respiratory chain components and redox balancing enzymes. In global knockouts, independently of their background, the metabolism of lipid, nucleic acid, and amino acids is inhibited, respiratory chain components are down-regulated, inflammatory response and regulation of chromosomal replication are enhanced. Decrease in glucokinase and lathosterol oxidase and elevation of mucin-13 and S100A10 are observed in all Atp7b mutant strains and reflect the extent of liver injury. The magnitude of proteomic changes in Atp7b
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.