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Nevertheless, inadequacy of existing FRDA-cardiac cellular models limited cardiomyopathy studies.
2
Conclusions: DFP modulated iron homeostasis in FRDA-hiPSC-cardiomyocytes and effectively relieved stress-stimulation related to cardiomyopathy.
3
Linkage data indicate that FRDA is at less than 1 cM from both markers.
4
FRDA is also associated with cognitive impairments.
5
Methods: Healthy control and FRDA patient-specific hPSC-hvCMs were derived by directed differentiation using a small molecule-based protocol reported previously.
6
Our aim was to investigate the changes in the cognitive functioning of FRDA patients over an average eight-year timeframe.
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Methods: Twenty-nine FRDA patients who had been part of the sample of a previous study participated in the present study.
8
The YACs were used to search for new polymorphic markers needed to map FRDA precisely with respect to the cloned segment.
9
High evolutionary conservation of frataxin across species has enabled the development of disease models of FRDA in various unicellular and multicellular organisms.
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Conclusions: Our study has demonstrated for the first time that patients with FRDA experience a significant decline over time in several cognitive domains.
11
We discuss the current hypotheses on the role of oxidative stress in FRDA and review some of the existing animal and cellular models.
12
Precise linkage mapping of the Friedreich ataxia locus (FRDA) in 9q13-q21 should lead to the isolation of the defective gene by positional cloning.