ArticleJournal of cellular and molecular medicine2015
Laser ablation inductively coupled plasma mass spectrometry imaging of metals in experimental and clinical Wilson's disease.
Article in Journal of cellular and molecular medicine, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 52 citations in OpenAlex.
- LASER ablation inductively coupled plasma mass spectrometry enables the recognition of new patterns in metal-related diseases.Scientific reports · 2025Article
- Modern Bioimaging Techniques for Elemental Tissue Analysis: Key Parameters, Challenges and Medical Impact.Molecules (Basel, Switzerland) · 2025Review
- Line-dropped gelatin multi-element calibration standards in LA-ICP-MS: a statistically verifying comparison with cryosectioned homogenized lung and liver as matrix-matched calibration standards and as corresponding reference materials.Analytical sciences : the international journal of the Japan Society for Analytical Chemistry · 2025Article
- Current Management of Neurological Wilson's Disease.Tremor and other hyperkinetic movements (New York, N.Y.) · 2025Review
- Ovaries of estrogen receptor 1-deficient mice show iron overload and signs of aging.Frontiers in endocrinology · 2024Article
- Quantitative bioimaging of copper in frozen liver specimens from cats using laser ablation-inductively coupled plasma-mass spectrometry.Journal of feline medicine and surgery · 2023Article
- Mass Spectrometry Imaging for Spatial Chemical Profiling of Vegetative Parts of Plants.Plants (Basel, Switzerland) · 2022Review
- Analyzing the Therapeutic Efficacy of Bis-Choline-Tetrathiomolybdate in theBiomedicines · 2021Article
- Accurate Measurement of Copper Overload in an Experimental Model of Wilson Disease by Laser Ablation Inductively Coupled Plasma Mass Spectrometry.Biomedicines · 2020Article
- Determination of copper poisoning in Wilson's disease using laser ablation inductively coupled plasma mass spectrometry.Annals of translational medicine · 2019Review
- Software solutions for evaluation and visualization of laser ablation inductively coupled plasma mass spectrometry imaging (LA-ICP-MSI) data: a short overview.Journal of cheminformatics · 2019Review
- Across the spectrum: integrating multidimensional metal analytics for in situ metallomic imaging.Metallomics : integrated biometal science · 2019Review
- Quantification of liver iron overload disease with laser ablation inductively coupled plasma mass spectrometry.BMC medical imaging · 2018Article
- Animal models of Wilson disease.Journal of neurochemistry · 2018Review
- Brain copper storage after genetic long-term correction in a mouse model of Wilson disease.Neurology. Genetics · 2018Article
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Authors and funding
10 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Wilson's disease is an autosomal recessive disorder in which the liver does not properly release copper into bile, resulting in prominent copper accumulation in various tissues. Affected patients suffer from hepatic disorders and severe neurological defects. Experimental studies in mutant mice in which the copper-transporting ATPase gene (Atp7b) is disrupted revealed a drastic, time-dependent accumulation of hepatic copper that is accompanied by formation of regenerative nodes resembling cirrhosis. Therefore, these mice represent an excellent exploratory model for Wilson's disease. However, the precise time course in hepatic copper accumulation and its impact on other trace metals within the liver is yet poorly understood. We have recently established novel laser ablation inductively coupled plasma mass spectrometry protocols allowing quantitative metal imaging in human and murine liver tissue with high sensitivity, spatial resolution, specificity and quantification ability. By use of these techniques, we here aimed to comparatively analyse hepatic metal content in wild-type and Atp7b deficient mice during ageing. We demonstrate that the age-dependent accumulation of hepatic copper is strictly associated with a simultaneous increase in iron and zinc, while the intrahepatic concentration and distribution of other metals or metalloids is not affected. The same findings were obtained in well-defined human liver samples that were obtained from patients suffering from Wilson's disease. We conclude that in Wilson's disease the imbalances of hepatic copper during ageing are closely correlated with alterations in intrahepatic iron and zinc content.
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