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@ARTICLE{Osterode:472642,
author = {Osterode, Wolf and Falkenberg, Gerald and Wrba, Fritz},
title = {{C}opper and {T}race {E}lements in {G}allbladder form
{P}atients with {W}ilson’s {D}isease {I}maged and
{D}etermined by {S}ynchrotron {X}-ray {F}luorescence},
journal = {Journal of imaging},
volume = {7},
number = {12},
issn = {2313-433X},
address = {Basel},
publisher = {MDPI},
reportid = {PUBDB-2021-05141},
pages = {261},
year = {2021},
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},
cin = {FS-PETRA-S},
ddc = {004},
cid = {I:(DE-H253)FS-PETRA-S-20210408},
pnm = {633 - Life Sciences – Building Blocks of Life: Structure
and Function (POF4-633)},
pid = {G:(DE-HGF)POF4-633},
experiment = {EXP:(DE-H253)D-L-20150101},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:34940728},
UT = {WOS:000738105200001},
doi = {10.3390/jimaging7120261},
url = {https://bib-pubdb1.desy.de/record/472642},
}