Journal Article

PUBDB-2021-05141

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Copper and Trace Elements in Gallbladder form Patients with Wilson’s Disease Imaged and Determined by Synchrotron X-ray Fluorescence

Osterode, W. (Corresponding author) ; Falkenberg, G.DESY* ; Wrba, F.

2021
MDPI Basel

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Please use a persistent id in citations: doi:10.3390/jimaging7120261  doi:10.3204/PUBDB-2021-05141

Abstract: Investigations about suspected tissue alterations and the role of gallbladder in Wilson’sdisease (WD)—an inherited genetic disease with impaired copper metabolism—are rare. Therefore,tissue from patients with genetically characterised WD was investigated by microscopic synchrotronX-ray fluorescence (SRXRF). For two-dimensional imaging and quantification of elements, X-rayspectra were peak-fitted, and the net peak intensities were normalised to the intensity of the incomingmonochromatic beam intensity. Concentrations were calculated by fundamental parameter-basedprogram 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 theseelements were increased compared to gallbladder tissue from controls. Cu and Zn and Fe in WD-GBwere mostly found to be enhanced in the epithelium. We documented a significant linear relationshipwith 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 andsulphur let us assume that metallothioneins, which are sulphur-rich proteins, are increased too. Ourdata let us suggest that the WD gallbladder is the first in the gastrointestinal tract to reabsorb metalsto prevent oxidative damage caused by metal toxicity

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Contributing Institute(s):

1. PETRA-S (FS-PETRA-S)

Research Program(s):

1. 633 - Life Sciences – Building Blocks of Life: Structure and Function (POF4-633) (POF4-633)

Experiment(s):

1. DORIS Beamline L (DORIS III)

Appears in the scientific report 2021

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