| First Author | Jacquier A | Year | 2009 |
| Journal | Hum Mol Genet | Volume | 18 |
| Issue | 12 | Pages | 2127-39 |
| PubMed ID | 19304783 | Mgi Jnum | J:148544 |
| Mgi Id | MGI:3845692 | Doi | 10.1093/hmg/ddp136 |
| Citation | Jacquier A, et al. (2009) Astrocytic protection of spinal motor neurons but not cortical neurons against loss of Als2/alsin function. Hum Mol Genet 18(12):2127-39 |
| abstractText | Three neurodegenerative diseases affecting upper and/or lower motor neurons have been associated with loss of ALS2/Alsin function: juvenile amyotrophic lateral sclerosis, primary lateral sclerosis and infantile-onset ascending hereditary spastic paralysis. The distinct neuronal vulnerability and the role of glia in these diseases remains, however, unclear. We here demonstrate that alsin-depleted spinal motor neurons can be rescued from defective survival and axon growth by co-cultured astrocytes. The astrocytic rescue is mediated by a soluble protective factor rather than by cellular contact. Cortical neurons are intrinsically as vulnerable to alsin depletion as spinal motor neurons but cannot be rescued by co-cultured astrocytes. To our knowledge, these data provide the first example of non-cell-autonomous glial effects in a recessive form of motor neuron disease and a potential rationale for the higher vulnerability of upper versus lower motor neurons in ALS2/Alsin-linked disorders. |
