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Scientific News

FARA funds research progress

In this section, you will find the most recent FA research publications, many of which are funded by FARA, as well as information on upcoming conferences and symposiums. You can search for articles by date using the archive box in the right hand column. To locate FARA Funded or Supported Research, click the hyperlink in the right hand column. You may also search for specific content using key words or phrases in the search button at the top right of your screen. Please be sure to visit other key research sections of our website for information on FARA’s Grant Program and the Treatment Pipeline.


Electrocardiogram in Friedreich's ataxia: A short-term surrogate endpoint for treatment efficacy

This study describes the case of a 21-year-old patient affected by Friedreich's ataxia on wheel-chair, with initial cardiac involvement and electrocardiographic features characterized by thiamine treatment-related negative T wave and QTc variations. The authors discuss plausible physiopathology and potential ECG role implications as an intermediate marker of treatment response in future clinical trials considering patients affected by Friedreich's ataxia.

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Epicardial ablation for ventricular tachycardia in Freidreich's ataxia cardiomyopathy

This work presents the first case of successful epicardial ablation of an electrical storm in a 55-year-old male patient with Friedreich’s ataxia, with advanced neurological involvement and dilated cardiomyopathy.

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An Overview of the Ferroptosis Hallmarks in Friedreich's Ataxia

Friedreich's ataxia (FRDA) is caused by reduced levels of frataxin, a mitochondrial protein involved in the synthesis of iron-sulfur clusters, leading to iron accumulation at the mitochondrial level, uncontrolled production of reactive oxygen species and lipid peroxidation. These features are also common to ferroptosis, an iron-mediated type of cell death triggered by accumulation of lipoperoxides with distinct morphological and molecular characteristics with respect to other known cell deaths. Even though ferroptosis has been associated with various neurodegenerative diseases including FRDA, the mechanisms leading to disease onset/progression have not been demonstrated yet. Here the authors describe the molecular alterations occurring in FRDA that overlap with those characterizing ferroptosis. The study of ferroptotic pathways is necessary for the understanding of FRDA pathogenesis, and anti-ferroptotic drugs could be envisaged as therapeutic strategies to cure FRDA.

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Oxidative stress modulates rearrangement of endoplasmic reticulum-mitochondria contacts and calcium dysregulation in a Friedreich's ataxia model

Endoplasmic Reticulum-mitochondria associated membranes (MAMs) are inter-organelle structures involved in the regulation of essential cellular processes, including lipid metabolism and calcium signaling. The present study analyzes calcium management and integrity of MAMs in both unicellular and multicellular models of Friedreich’s ataxia (FRDA). The authors observed that the function of MAMs is compromised in a cellular model of FRDA, which was improved upon treatment with antioxidants. In agreement, promoting mitochondrial calcium uptake was sufficient to restore several defects caused by frataxin deficiency in Drosophila Melanogaster. Remarkably, these findings describe for the first time frataxin as a member of the protein network of MAMs, where it interacts with two of the main proteins implicated in endoplasmic reticulum-mitochondria communication. These results suggest a new role of frataxin, indicate that FRDA goes beyond mitochondrial defects and highlight MAMs as novel therapeutic candidates to improve patients' conditions.

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Aerobic Exercises Induce Antioxidant Pathways Activation in Rats

Aerobic exercises induce adaptations that improve physiological function. However, aerobic exercises, oxidative reproduction may lead to injury and other health issues such as adverse cardiac effects. The aim of this study is to evaluate the effect of aerobic exercises on protein expression change in the heart left ventricle to determine the advantages and disadvantages related to this mode of exercise. Male Wistar rats were randomized into two groups; trained (T) and control (C). Animals from T group were trained for 8 weeks, and then 2D LC-MS/MS iTRAQ method was used for extracting and analyzing the left ventricular proteins. Certain proteins that were highlighted in the special process were selected for further analysis via protein-protein interaction network (PPI) method. The identified proteins were enriched via gene ontology (GO) to find biological terms. The authors identify five overexpressed antioxidant proteins in T group compared with C group including extracellular superoxide dismutase [Cu-Zn], Frataxin, protein kinase C delta type, STE20/SPS1-related proline-alanine-rich protein kinase, and amyloid-beta A4 protein. Findings indicate that catalase and insulin are two exercise-related proteins. However, they were not included in the significant differentially expressed proteins. Finally it was found that enhancement of antioxidative activity is a direct effect of aerobic exercises.

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