Nanoscopic X-ray imaging and quantification of the iron cellular architecture within single fibroblasts of Friedreich's ataxia patients

Nanoscopic X-ray imaging and quantification of the iron cellular architecture within single fibroblasts of Friedreich's ataxia patients

Friedreich's ataxia (FRDA) is a neurodegenerative disease characterized by an increase in intracytoplasmic iron concentration. Here the nanoscale iron distribution within single fibroblasts from FRDA patients was investigated using synchrotron-radiation-based nanoscopic X-ray fluorescence and X...

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Veröffentlicht in:Journal of synchrotron radiation. - 1994. - 27(2020), Pt 1 vom: 01. Jan., Seite 185-198
1. Verfasser: De Samber, Björn (VerfasserIn)
Weitere Verfasser: Vanden Berghe, Tom, Meul, Eline, Bauters, Stephen, Seyrich, Martin, Smet, Joél, De Paepe, Boel, da Silva, Julio Cesar, Bohic, Sylvain, Cloetens, Peter, Van Coster, Rudy, Vandenabeele, Peter, Vincze, Laszlo
Format: Online-Aufsatz
Sprache:English
Veröffentlicht: 2020
Zugriff auf das übergeordnete Werk:Journal of synchrotron radiation
Schlagworte:Journal Article Friedreich's ataxia imaging iron nano-XRF quantification Carbon 7440-44-0 Iron E1UOL152H7
Beschreibung
Zusammenfassung:Friedreich's ataxia (FRDA) is a neurodegenerative disease characterized by an increase in intracytoplasmic iron concentration. Here the nanoscale iron distribution within single fibroblasts from FRDA patients was investigated using synchrotron-radiation-based nanoscopic X-ray fluorescence and X-ray in-line holography at the ID16A nano-imaging beamline of the ESRF. This unique probe was deployed to uncover the iron cellular two-dimensional architecture of freeze-dried FRDA fibroblasts. An unsurpassed absolute detection capability of 180 iron atoms within a 30 nm × 50 nm nanoscopic X-ray beam footprint was obtained using state-of-the-art X-ray focusing optics and a large-solid-angle detection system. Various micrometre-sized iron-rich organelles could be revealed for the first time, tentatively identified as endoplasmic reticulum, mitochondria and lysosomes. Also a multitude of nanoscopic iron hot-spots were observed in the cytosol, interpreted as chaperoned iron within the fibroblast's labile iron pool. These observations enable new hypotheses on the storage and trafficking of iron in the cell and ultimately to a better understanding of iron-storage diseases such as Friedreich's ataxia
Beschreibung:Date Completed 08.07.2020
Date Revised 08.07.2020
published: Print-Electronic
Citation Status MEDLINE
ISSN:1600-5775
DOI:10.1107/S1600577519015510