First person – Claire Montgomery and Lili Salinas

TL;DR

This paper presents a study on a mouse model of Friedreich's ataxia and evaluates the effectiveness of a treatment approach.

Contribution

The study provides a robust behavioral assessment of a mouse model and challenges the efficacy of NRF2 induction as a treatment.

Findings

The inducible Friedreich's ataxia mouse model was behaviorally assessed.

NRF2 induction did not improve outcomes in the mouse model.

The findings question the therapeutic potential of NRF2 activation in this disease.

Abstract

First Person is a series of interviews with the first authors of a selection of papers published in Disease Models & Mechanisms, helping researchers promote themselves alongside their papers. Claire Montgomery and Lili Salinas are co-first authors on ‘
Robust behavioral assessment of the inducible Friedreich's ataxia mouse does not show improvement with NRF2 induction’, published in DMM. Claire is a staff research associate in the lab of Drs Elena N. Dedkova and Gino Cortopassi at University of California, Davis, Davis, CA, USA, investigating mitochondria and their role in different metabolic diseases, drug development for rare mitochondrial diseases and mitochondrial toxicity. Lili is a PhD student in the same lab, investigating rare mitochondrial diseases, their pathophysiology and in vivo pharmacology in such diseases.

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