Schwann cell JUN expression worsens motor performance in an amyotrophic lateral sclerosis mouse model.

Autor:	Cabeza-Fernández S; Instituto de Investigación Biomédica y Sanitaria de Alicante (ISABIAL), Alicante, Spain.; Molecular control of neuronal axon myelination laboratory, Instituto de Neurociencias UMH-CSIC, Sant Joan d'Alacant, Spain., Hernández-Rojas R; Instituto de Investigación Biomédica y Sanitaria de Alicante (ISABIAL), Alicante, Spain.; Molecular control of neuronal axon myelination laboratory, Instituto de Neurociencias UMH-CSIC, Sant Joan d'Alacant, Spain., Casillas-Bajo A; Instituto de Investigación Biomédica y Sanitaria de Alicante (ISABIAL), Alicante, Spain.; Molecular control of neuronal axon myelination laboratory, Instituto de Neurociencias UMH-CSIC, Sant Joan d'Alacant, Spain., Patel N; Instituto de Investigación Biomédica y Sanitaria de Alicante (ISABIAL), Alicante, Spain.; Molecular control of neuronal axon myelination laboratory, Instituto de Neurociencias UMH-CSIC, Sant Joan d'Alacant, Spain., de la Fuente AG; Instituto de Investigación Biomédica y Sanitaria de Alicante (ISABIAL), Alicante, Spain.; Molecular control of neuronal axon myelination laboratory, Instituto de Neurociencias UMH-CSIC, Sant Joan d'Alacant, Spain., Cabedo H; Instituto de Investigación Biomédica y Sanitaria de Alicante (ISABIAL), Alicante, Spain.; Molecular control of neuronal axon myelination laboratory, Instituto de Neurociencias UMH-CSIC, Sant Joan d'Alacant, Spain., Gomez-Sanchez JA; Instituto de Investigación Biomédica y Sanitaria de Alicante (ISABIAL), Alicante, Spain.; Molecular control of neuronal axon myelination laboratory, Instituto de Neurociencias UMH-CSIC, Sant Joan d'Alacant, Spain.
Jazyk:	angličtina
Zdroj:	Glia [Glia] 2024 Dec; Vol. 72 (12), pp. 2178-2189. Date of Electronic Publication: 2024 Aug 16.
DOI:	10.1002/glia.24604
Abstrakt:	Amyotrophic lateral sclerosis is a devastating neurodegenerative disease characterized by motor neuron death and distal axonopathy. Despite its clinical severity and profound impact in the patients and their families, many questions about its pathogenesis remain still unclear, including the role of Schwann cells and axon-glial signaling in disease progression. Upon axonal injury, upregulation of JUN transcription factor promotes Schwann cell reprogramming into a repair phenotype that favors axon regrowth and neuronal survival. To study the potential role of repair Schwann cells on motoneuron survival in amyotrophic lateral sclerosis, we generated a mouse line that over-expresses JUN in the Schwann cells of the SOD1 G93A mutant, a mouse model of this disease. Then, we explored disease progression by evaluating survival, motor performance and histology of peripheral nerves and spinal cord of these mice. We found that Schwann cell JUN overexpression does not prevent axon degeneration neither motor neuron death in the SOD1 G93A mice. Instead, it induces a partial demyelination of medium and large size axons, worsening motor performance and resulting in more aggressive disease phenotype. (© 2024 The Author(s). GLIA published by Wiley Periodicals LLC.)
Databáze:	MEDLINE
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