Copper and Trace Elements in Gallbladder form Patients with Wilson’s Disease Imaged and Determined by Synchrotron X-ray Fluorescence
Investigations about suspected tissue alterations and the role of gallbladder in Wilson’s disease (WD)—an inherited genetic disease with impaired copper metabolism—are rare. Therefore, tissue from patients with genetically characterised WD was investigated by microscopic synchrotron X-ray fluorescen...
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| Format: | Article |
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MDPI AG
2021-12-01
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| Series: | Journal of Imaging |
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| Online Access: | https://www.mdpi.com/2313-433X/7/12/261 |
| _version_ | 1827671905094598656 |
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| author | Wolf Osterode Gerald Falkenberg Fritz Wrba |
| author_facet | Wolf Osterode Gerald Falkenberg Fritz Wrba |
| author_sort | Wolf Osterode |
| collection | DOAJ |
| description | Investigations about suspected tissue alterations and the role of gallbladder in Wilson’s disease (WD)—an inherited genetic disease with impaired copper metabolism—are rare. Therefore, tissue from patients with genetically characterised WD was investigated by microscopic synchrotron X-ray fluorescence (µSRXRF). For two-dimensional imaging and quantification of elements, X-ray spectra were peak-fitted, and the net peak intensities were normalised to the intensity of the incoming monochromatic beam intensity. Concentrations were calculated by fundamental parameter-based program quant and external standardisation. Copper (Cu), zinc (Zn) and iron (Fe) along with sulphur (S) and phosphorus (P) mappings could be demonstrated in a near histological resolution. All these elements were increased compared to gallbladder tissue from controls. Cu and Zn and Fe in WD-GB were mostly found to be enhanced in the epithelium. We documented a significant linear relationship with Cu, Zn and sulphur. Concentrations of Cu/Zn were roughly 1:1 while S/Cu was about 100:1, depending on the selected areas for investigation. The significant linear relationship with Cu, Zn and sulphur let us assume that metallothioneins, which are sulphur-rich proteins, are increased too. Our data let us suggest that the WD gallbladder is the first in the gastrointestinal tract to reabsorb metals to prevent oxidative damage caused by metal toxicity. |
| first_indexed | 2024-03-10T03:50:20Z |
| format | Article |
| id | doaj.art-fe16f24c833b4ef18ca66b1296387e8e |
| institution | Directory Open Access Journal |
| issn | 2313-433X |
| language | English |
| last_indexed | 2024-03-10T03:50:20Z |
| publishDate | 2021-12-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Imaging |
| spelling | doaj.art-fe16f24c833b4ef18ca66b1296387e8e2023-11-23T09:00:39ZengMDPI AGJournal of Imaging2313-433X2021-12-0171226110.3390/jimaging7120261Copper and Trace Elements in Gallbladder form Patients with Wilson’s Disease Imaged and Determined by Synchrotron X-ray FluorescenceWolf Osterode0Gerald Falkenberg1Fritz Wrba2Universitätsklinik für Innere Medizin II, Medical University of Vienna, A-1090 Vienna, AustriaDeutsches Elektronen-Synchrotron (DESY), Photon Science, D-22603 Hamburg, GermanyKlinisches Institut für Klinische Pathologie, Medical University of Vienna, A-1090 Vienna, AustriaInvestigations about suspected tissue alterations and the role of gallbladder in Wilson’s disease (WD)—an inherited genetic disease with impaired copper metabolism—are rare. Therefore, tissue from patients with genetically characterised WD was investigated by microscopic synchrotron X-ray fluorescence (µSRXRF). For two-dimensional imaging and quantification of elements, X-ray spectra were peak-fitted, and the net peak intensities were normalised to the intensity of the incoming monochromatic beam intensity. Concentrations were calculated by fundamental parameter-based program quant and external standardisation. Copper (Cu), zinc (Zn) and iron (Fe) along with sulphur (S) and phosphorus (P) mappings could be demonstrated in a near histological resolution. All these elements were increased compared to gallbladder tissue from controls. Cu and Zn and Fe in WD-GB were mostly found to be enhanced in the epithelium. We documented a significant linear relationship with Cu, Zn and sulphur. Concentrations of Cu/Zn were roughly 1:1 while S/Cu was about 100:1, depending on the selected areas for investigation. The significant linear relationship with Cu, Zn and sulphur let us assume that metallothioneins, which are sulphur-rich proteins, are increased too. Our data let us suggest that the WD gallbladder is the first in the gastrointestinal tract to reabsorb metals to prevent oxidative damage caused by metal toxicity.https://www.mdpi.com/2313-433X/7/12/261Wilson’s diseasegallbladdersynchrotron X-ray fluorescencecopperzincsulphur |
| spellingShingle | Wolf Osterode Gerald Falkenberg Fritz Wrba Copper and Trace Elements in Gallbladder form Patients with Wilson’s Disease Imaged and Determined by Synchrotron X-ray Fluorescence Journal of Imaging Wilson’s disease gallbladder synchrotron X-ray fluorescence copper zinc sulphur |
| title | Copper and Trace Elements in Gallbladder form Patients with Wilson’s Disease Imaged and Determined by Synchrotron X-ray Fluorescence |
| title_full | Copper and Trace Elements in Gallbladder form Patients with Wilson’s Disease Imaged and Determined by Synchrotron X-ray Fluorescence |
| title_fullStr | Copper and Trace Elements in Gallbladder form Patients with Wilson’s Disease Imaged and Determined by Synchrotron X-ray Fluorescence |
| title_full_unstemmed | Copper and Trace Elements in Gallbladder form Patients with Wilson’s Disease Imaged and Determined by Synchrotron X-ray Fluorescence |
| title_short | Copper and Trace Elements in Gallbladder form Patients with Wilson’s Disease Imaged and Determined by Synchrotron X-ray Fluorescence |
| title_sort | copper and trace elements in gallbladder form patients with wilson s disease imaged and determined by synchrotron x ray fluorescence |
| topic | Wilson’s disease gallbladder synchrotron X-ray fluorescence copper zinc sulphur |
| url | https://www.mdpi.com/2313-433X/7/12/261 |
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