Supplementary figures (Figure S1, S2, S3, and S4). (PDF 2135 kb

Berit Powers

Fumito Endo

Hidemi Misawa

Jin Shijie

John Ravits

Jurate Lasiene

Koji Yamanaka

Noriko Fujimori-Tonou

Okiru Komine

Philip Horner

Seiji Watanabe

Acta Neuropathologica Communications

PubMed

Crossref

Neuregulin 1 confers neuroprotection in SOD1-linked amyotrophic lateral sclerosis mice via restoration of C-boutons of spinal motor neurons

Springer - Publisher Connector

Jurate Lasiene (3502175)

Okiru Komine (66653)

Noriko Fujimori-Tonou (3502184)

Berit Powers (3502172)

Fumito Endo (3502181)

Seiji Watanabe (356430)

Jin Shijie (3502187)

John Ravits (3502178)

Philip Horner (3502169)

Hidemi Misawa (145997)

Koji Yamanaka (217338)

The Francis Crick Institute

Additional file 1: of Neuregulin 1 confers neuroprotection in SOD1-linked amyotrophic lateral sclerosis mice via restoration of C-boutons of spinal motor neurons

INTRODUCTION: Increasing evidence implicates the role of the cell types surrounding motor neurons, such as interneurons and glial cells, in non-cell autonomous neurodegeneration of amyotrophic lateral sclerosis (ALS). C-boutons, the large cholinergic synapses that innervate spinal α-motor neurons to control their excitability, are progressively lost from motor neurons in both human ALS and mutant Cu/Zn superoxide dismutase 1 (SOD1)-ALS mice. Neuregulin-1 (NRG1), a trophic factor implicated in neural development, transmission, and synaptic plasticity, has been reported to localize in the synapse of C-boutons. However, the roles of NRG1 in maintenance of motor neuron health and activity, as well as the functional consequences of its alteration in motor neuron disease, are not fully understood. RESULTS: NRG1 was localized to the post-synaptic face of C-boutons and its expression was significantly lost in SOD1-ALS mice and human ALS patients. Losses of NRG1 expression and C-boutons occurred almost contemporaneously in SOD1-ALS mice. In addition, expressions of ErbB3 and ErbB4, receptors for NRG1, were reduced in the motor neurons of SOD1-ALS mice. Furthermore, viral-mediated delivery of type III-NRG1 to the spinal cord restored the number of C-boutons and extended the survival time of SOD1-ALS mice. CONCLUSIONS: These results suggest that maintenance of NRG1-ErbB4/3 axis by supplementation of NRG1 confers neuroprotection in motor neuron disease, partly through the maintenance of C-boutons of spinal motor neurons

Lasiene, Jurate

Komine, Okiru

Fujimori-Tonou, Noriko

Powers, Berit

Endo, Fumito

Watanabe, Seiji

Shijie, Jin

Ravits, John

Horner, Philip

Misawa, Hidemi

Yamanaka, Koji

eScholarship - University of California

English

Neuregulin 1 confers neuroprotection in SOD1-linked amyotrophic lateral sclerosis mice via restoration of C-boutons of spinal motor neurons.

http://dx.doi.org/10.1186/s40478-016-0286-7

Neuregulin 1 confers neuroprotection in SOD1-linked amyotrophic lateral sclerosis mice via restoration of C-boutons of spinal motor neurons

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Springer - Publisher Connector

The Francis Crick Institute

eScholarship - University of California