| First Author | Lee N | Year | 2019 |
| Journal | Exp Neurol | Volume | 317 |
| Pages | 202-205 | PubMed ID | 30902524 |
| Mgi Jnum | J:283614 | Mgi Id | MGI:6355762 |
| Doi | 10.1016/j.expneurol.2019.03.009 | Citation | Lee N, et al. (2019) Muscle ciliary neurotrophic factor receptor alpha helps maintain choline acetyltransferase levels in denervated motor neurons following peripheral nerve lesion. Exp Neurol 317:202-205 |
| abstractText | Systemic ciliary neurotrophic factor (CNTF) administration protects motor neurons from denervating diseases and lesions but produces non-neuromuscular side effects. Therefore, CNTF related therapeutics will need to specifically target motor neuron protective receptor mechanisms. Expression of the essential ligand binding subunit of the CNTF receptor, CNTF receptor alpha (CNTFRalpha), is induced in skeletal muscle by denervating lesion and in human denervating diseases. We show here, with muscle-specific in vivo genetic disruption, that muscle CNTFRalpha makes an essential/non-redundant contribution to maintaining choline acetyltransferase levels in denervated motor neurons following nerve crush, suggesting the muscle CNTFRalpha induction is an endogenous denervation-induced neuroprotective response that could be enhanced to treat nerve lesion and denervating diseases. Notably, unlike motor neuron gene expression, skeletal muscle gene expression can be specifically targeted with human gene therapy vectors already approved for market. |
