Astrocytic protection of spinal motor neurons but not cortical neurons against loss of Als2/alsin function

Hum Mol Genet. 2009 Jun 15;18(12):2127-39. doi: 10.1093/hmg/ddp136. Epub 2009 Mar 20.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Astrocytes / metabolism*
  • Cell Line
  • Cells, Cultured
  • Cerebral Cortex / cytology*
  • Cerebral Cortex / metabolism
  • Cerebral Cortex / physiopathology
  • Disease Models, Animal
  • Guanine Nucleotide Exchange Factors / genetics
  • Guanine Nucleotide Exchange Factors / metabolism*
  • Humans
  • Mice
  • Mice, Knockout
  • Motor Neuron Disease / genetics
  • Motor Neuron Disease / metabolism*
  • Motor Neuron Disease / physiopathology
  • Motor Neurons / metabolism*
  • Spine / cytology*
  • Spine / metabolism
  • Spine / physiopathology

Substances

  • Als2 protein, mouse
  • Guanine Nucleotide Exchange Factors