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Open Access Highly Accessed Research article

Trophic and proliferative effects of Shh on motor neurons in embryonic spinal cord culture from wildtype and G93A SOD1 mice

Xiaoxing Ma1, Patrick Turnbull12, Randy Peterson13 and John Turnbull1*

Author Affiliations

1 Department of Medicine, McMaster University, 1200 Main St West, Hamilton, ON L8N 3Z5, Canada

2 Present address: Faculty of Applied Health Sciences, Brock University, 500 Glenridge Ave, St. Catharines, ON L2S 3A1, Canada

3 Present address: Biotechnology Group, Activation Laboratories, 1336 Sandhill Drive, Ancaster, ON L9G 4 V5, Canada

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BMC Neuroscience 2013, 14:119  doi:10.1186/1471-2202-14-119

Published: 11 October 2013

Abstract

Background

The developmental morphogen sonic hedgehog (Shh) may continue to play a trophic role in the support of terminally-differentiated motor neurons, of potential relevance to motor neuron disease. In addition, it may support the proliferation and differentiation of endogenous stem cells along motor neuronal lineages. As such, we have examined the trophic and proliferative effects of Shh supplementation or Shh antagonism in embryonic spinal cord cell cultures derived from wildtype or G93A SOD1 mice, a mouse model of amyotrophic lateral sclerosis.

Results

Shh supported survival, and stimulated growth of motor neurons, neurite outgrowth, and neurosphere formation in primary culture derived from both G93A SOD1 and WT mice. Shh increased the percentage of ciliated motor neurons, especially in G93A SOD1 culture. Shh-treated cultures showed increased neuronal proliferation compared to controls and especially cyclopamine treated cultures, from G93A SOD1 and WT mice. Moreover, Shh enhanced cell survival and differentiation of motor neuron precursors in WT culture.

Conclusions

Shh is neurotrophic to motor neurons and has mitogenic effects in WT and mSOD1 G93A culture in vitro.

Keywords:
Sonic hedgehog; Primary cilium; Motor neuron; Amyotrophic lateral sclerosis