% IMPORTANT: The following is UTF-8 encoded. This means that in the presence
% of non-ASCII characters, it will not work with BibTeX 0.99 or older.
% Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or
% “biber”.
@ARTICLE{Reetz:1043578,
author = {Reetz, Kathrin and Lischewski, Stella A and Dogan, Imis and
Didszun, Claire and Pishnamaz, Miguel and Konrad, Kerstin
and Marx-Schütt, Katharina and Farmer, Jennifer and Lynch,
David R and Corben, Louise A and Pandolfo, Massimo and
Schulz, Jörg B and Costa, Ana Sofia and Romanzetti, Sandro
and Dadsena, Ravi and Praster, Maximillian and Clavel,
Thomas and Jankowski, Vera and Jankowski, Joachim and Pabst,
Oliver and Marx, Nikolaus and Möllmann, Julia and Jacobsen,
Malte and Dukart, Juergen and Eickhoff, Simon and Hilgers,
Ralf-Dieter},
title = {{F}riedreich's ataxia—a rare multisystem disease},
journal = {The lancet},
volume = {24},
number = {7},
issn = {1474-4422},
address = {London},
publisher = {Lancet Publ. Group},
reportid = {FZJ-2025-02935},
pages = {614 - 624},
year = {2025},
abstract = {Friedreich's ataxia is a rare autosomal recessive
neurodegenerative disease. Most patients have a homozygous
GAA repeat expansion in the FXN gene, resulting in a
deficiency of the mitochondrial protein frataxin. Disease
onset occurs typically in adolescence but can vary widely,
ranging from early childhood to late adulthood. Friedreich's
ataxia is increasingly recognised as a multisystem disorder,
affecting not only the nervous system, but also the heart
and musculoskeletal system, and metabolism. Common
extraneural manifestations include cardiomyopathy, which is
the most common cause of mortality, and also scoliosis and
diabetes. Despite research advances, the phenotypical
heterogeneity of patients with Friedrich's ataxia remains
inadequately explained by current knowledge of the
underlying genetics. The approval of omaveloxolone by the US
Food and Drug Administration and the European Medicines
Agency has been a pharmacological milestone; however,
further research addressing complex interorgan interactions
is crucial for a better understanding of the multisystem
nature of Friedreich's ataxia and the development of
targeted treatment approaches.},
cin = {INM-7 / INM-11},
ddc = {610},
cid = {I:(DE-Juel1)INM-7-20090406 / I:(DE-Juel1)INM-11-20170113},
pnm = {5251 - Multilevel Brain Organization and Variability
(POF4-525)},
pid = {G:(DE-HGF)POF4-5251},
typ = {PUB:(DE-HGF)16},
pubmed = {40541211},
UT = {WOS:001520810300012},
doi = {10.1016/S1474-4422(25)00175-9},
url = {https://juser.fz-juelich.de/record/1043578},
}