Open AccessNanoscopic X‐ray imaging and quantification of the iron cellular architecture within single fibroblasts of Friedreich's ataxia patients
Author(s) -
De Samber Björn,
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
Publication year - 2020
Publication title -
journal of synchrotron radiation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.172
H-Index - 99
ISSN - 1600-5775
DOI - 10.1107/s1600577519015510
Subject(s) - nanoscopic scale , biophysics , synchrotron , ataxia , fluorescence , materials science , nanotechnology , synchrotron radiation , chemistry , biology , optics , physics , neuroscience
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.