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Publication : Decreased signalling of EphA4 improves functional performance and motor neuron survival in the SOD1<sup>G93A</sup> ALS mouse model.

First Author  Zhao J Year  2018
Journal  Sci Rep Volume  8
Issue  1 Pages  11393
PubMed ID  30061574 Mgi Jnum  J:269634
Mgi Id  MGI:6271651 Doi  10.1038/s41598-018-29845-1
Citation  Zhao J, et al. (2018) Decreased signalling of EphA4 improves functional performance and motor neuron survival in the SOD1(G93A) ALS mouse model. Sci Rep 8(1):11393
abstractText  Amyotrophic lateral sclerosis (ALS) is an untreatable, progressive, neurodegenerative disease specifically affecting motor neurons. Recently, the tyrosine kinase receptor EphA4 was directly implicated in ALS disease progression. We report that a long-lived mutated form of the EphA4 antagonist EphA4-Fc (mutEphA4-Fc), which blocks EphA4 binding to its ligands and inhibits its function, significantly improved functional performance in SOD1(G93A) ALS model mice, as assessed by rotarod and hind-limb grip strength tests. Further, heterozygous motor neuron-specific EphA4 gene deletion in SOD1(G93A) mice promoted significant improvement in functional performance during the disease course and a delay in disease onset relative to control mice. Importantly, mice in the heterozygous deletion group showed significantly improved survival of motor neurons and architecture of endplates of neuromuscular junctions compared with control and homozygous EphA4-deletion groups. Our novel results show that EphA4 signalling directly regulates motor neuron survival and that mutEphA4-Fc is a promising therapeutic candidate to slow disease progression in ALS.
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