Nxnl2 splicing results in dual functions in neuronal cell survival and maintenance of cell integrity. - Inserm - Institut national de la santé et de la recherche médicale Access content directly
Journal Articles Human Molecular Genetics Year : 2012

Nxnl2 splicing results in dual functions in neuronal cell survival and maintenance of cell integrity.

Marie-Laure Niepon
  • Function : Author
  • PersonId : 744480
  • IdHAL : mlniepon
Ying Yang
  • Function : Author
Bernd Kinzel
  • Function : Author
Pierre-Marie Lledo
José-Alain Sahel
Thierry Léveillard
  • Function : Correspondent author
  • PersonId : 852904

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Abstract

The rod-derived cone viability factors, RdCVF and RdCVF2, have potential therapeutical interests for the treatment of inherited photoreceptor degenerations. In the mouse lacking Nxnl2, the gene encoding RdCVF2, the progressive decline of the visual performance of the cones in parallel with their degeneration, arises due to the loss of trophic support from RdCVF2. In contrary, the progressive loss of rod visual function of the Nxnl2-/- mouse results from a decrease in outer segment length, mediated by a cell autonomous mechanism involving the putative thioredoxin protein RdCVF2L, the second spliced product of the Nxnl2 gene. This novel signaling mechanism extends to olfaction as shown by the progressive impairment of olfaction in aged Nxnl2-/- mice and the protection of olfactory neurons by RdCVF2. This study shows that Nxnl2 is a bi-functional gene involved in the maintenance of both the function and the viability of sensory neurons.

Domains

Genetics
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Dates and versions

inserm-00675213 , version 1 (01-03-2013)

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Céline Jaillard, Aurélie Mouret, Marie-Laure Niepon, Emmanuelle Clérin, Ying Yang, et al.. Nxnl2 splicing results in dual functions in neuronal cell survival and maintenance of cell integrity.. Human Molecular Genetics, 2012, 21 (10), pp.2298-311. ⟨10.1093/hmg/dds050⟩. ⟨inserm-00675213⟩
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