Astrocytic protection of spinal motor neurons but not cortical neurons against loss of Als2/alsin function.
Hum Mol Genet
; 18(12): 2127-39, 2009 Jun 15.
Article
en En
| MEDLINE
| ID: mdl-19304783
ABSTRACT
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.
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Columna Vertebral
/
Corteza Cerebral
/
Astrocitos
/
Enfermedad de la Neurona Motora
/
Factores de Intercambio de Guanina Nucleótido
/
Neuronas Motoras
Tipo de estudio:
Prognostic_studies
Límite:
Animals
/
Humans
Idioma:
En
Revista:
Hum Mol Genet
Asunto de la revista:
BIOLOGIA MOLECULAR
/
GENETICA MEDICA
Año:
2009
Tipo del documento:
Article
País de afiliación:
Francia