ADAR1 mediated regulation of neural crest derived melanocytes and Schwann cell development

Nadjet Gacem, Anthula Kavo, Lisa Zerad, Laurence Richard, Stephane Mathis, Raj P. Kapur, Melanie Parisot, Jeanne Amiel, Sylvie Dufour, Pierre de la Grange, Veronique Pingault, Jean Michel Vallat and Nadege Bondurand ()
Additional contact information
Nadjet Gacem: Universite Paris Descartes—Universite de Paris
Anthula Kavo: INSERM, U955, Equipe 06
Lisa Zerad: Universite Paris Descartes—Universite de Paris
Laurence Richard: Centre de Reference Neuropathies Peripheriques Rares
Stephane Mathis: Pellegrin Hospital
Raj P. Kapur: Seattle Children’s Hospital and University of Washington
Melanie Parisot: INSERM U1163 and INSERM US24/CNRS UMS3633
Jeanne Amiel: Universite Paris Descartes—Universite de Paris
Sylvie Dufour: INSERM, U955, Equipe 06
Pierre de la Grange: GenoSplice
Veronique Pingault: Universite Paris Descartes—Universite de Paris
Jean Michel Vallat: Centre de Reference Neuropathies Peripheriques Rares
Nadege Bondurand: Universite Paris Descartes—Universite de Paris

Nature Communications, 2020, vol. 11, issue 1, 1-14

Abstract: Abstract The neural crest gives rise to numerous cell types, dysfunction of which contributes to many disorders. Here, we report that adenosine deaminase acting on RNA (ADAR1), responsible for adenosine-to-inosine editing of RNA, is required for regulating the development of two neural crest derivatives: melanocytes and Schwann cells. Neural crest specific conditional deletion of Adar1 in mice leads to global depigmentation and absence of myelin from peripheral nerves, resulting from alterations in melanocyte survival and differentiation of Schwann cells, respectively. Upregulation of interferon stimulated genes precedes these defects, which are associated with the triggering of a signature resembling response to injury in peripheral nerves. Simultaneous extinction of MDA5, a key sensor of unedited RNA, rescues both melanocytes and myelin defects in vitro, suggesting that ADAR1 safeguards neural crest derivatives from aberrant MDA5-mediated interferon production. We thus extend the landscape of ADAR1 function to the fields of neural crest development and disease.

Date: 2020
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Persistent link: https://EconPapers.repec.org/RePEc:nat:natcom:v:11:y:2020:i:1:d:10.1038_s41467-019-14090-5

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DOI: 10.1038/s41467-019-14090-5

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