Astrocytic protection of spinal motor neurons but not cortical neurons against loss of Als2/alsin function
| Autor: | Georg Haase, Delphine Bohl, Arnaud Jacquier, Sarah Bellouze, Stéphane Blanchard |
|---|---|
| Rok vydání: | 2009 |
| Předmět: |
Juvenile amyotrophic lateral sclerosis
Biology Cell Line Mice Genetics medicine Animals Guanine Nucleotide Exchange Factors Humans Motor Neuron Disease Axon Molecular Biology Cells Cultured Genetics (clinical) Primary Lateral Sclerosis Cerebral Cortex Mice Knockout Motor Neurons General Medicine Cortical neurons Anatomy Motor neuron Spine Axon growth Disease Models Animal medicine.anatomical_structure nervous system Astrocytes Neuroscience Function (biology) Spastic paralysis |
| Zdroj: | Human Molecular Genetics. 18:2127-2139 |
| ISSN: | 1460-2083 0964-6906 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|