Abstract
Peripheral neuropathies can have various origins, from genetic to acquired causes, and affect altogether a large group of people in the world. Current available therapies aim at helping the disease symptoms but not to correct or stop the development of the disease. Primary neuronal cultures represent an essential tool in the study of events related to peripheral neuropathies as they allow to isolate the affected cell types, often originating in complex tissues in which they account for only a few percentage of cells. They provide a powerful system to identifying or testing compounds with potential therapeutic effect in the treatment of those diseases. Friedreich’s ataxia is an autosomal recessive neurodegenerative disorder, which is characterized by a progressive spinocerebellar and sensory ataxia. Proprioceptive neurons of the dorsal root ganglia (DRG) are the primary affected cells. The disease is triggered by a mutation in the gene FXN which leads to a reduction of the frataxin protein. In order to study the neurophysiopathology of the disease at the cellular and molecular levels, we have established a model of primary cultures of DRG sensory neurons in which we induce the loss of the frataxin protein. With such a model we can alleviate the issues related to the complexity of DRG tissues and low amount of sensory neuron material in adult mouse. Hereby, we provide a protocol of detailed and optimized methods to obtain high yield of healthy mouse DRG sensory neuron in culture.
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Acknowledgments
We thank Aurélie Eisenmann and Alain Martelli for technical assistance. This work was supported by the US Friedreich Ataxia Research Alliance (to H.P.), the Association Française pour l’Ataxie de Friedreich (to H.P.) and the Fondation pour la Recherche Médicale (FRM grant number ECO20160736060 (to O.G.)). This study was supported by the grant ANR-10-LABX-0030-INRT, a French State fund managed by the Agence Nationale de la Recherche under the frame program Investissement d’Avenir ANR-10-IDEX-0002-02.
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Griso, O., Puccio, H. (2020). Primary Cultures of Pure Embryonic Dorsal Root Ganglia Sensory Neurons as a New Cellular Model for Friedreich’s Ataxia. In: Richard, GF. (eds) Trinucleotide Repeats. Methods in Molecular Biology, vol 2056. Humana, New York, NY. https://doi.org/10.1007/978-1-4939-9784-8_15
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DOI: https://doi.org/10.1007/978-1-4939-9784-8_15
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