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

De Samber, B., Vanden Berghe, T., Meul, E., Bauters, S., Seyrich, M., Smet, J., De Paepe, B., da Silva, J.C., Bohic, S., Cloetens, P., Van Coster, R., Vandenabeele, P. and Vincze, L. (2020), J. Synchrotron Rad.. doi:10.1107/S1600577519015510

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.
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.


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