ALS: astrocytes move in as deadly neighbors

  title={ALS: astrocytes move in as deadly neighbors},
  author={Jean-Pierre Julien},
  journal={Nature Neuroscience},
  • J. Julien
  • Published 1 May 2007
  • Biology
  • Nature Neuroscience
Amyotrophic lateral sclerosis (ALS) is characterized by the selective degeneration of motor neurons. Two independent studies in this issue show that astrocytes expressing a mutation in the enzyme superoxide dismutase can exacerbate motor neuron death, supporting previous suggestions that non-neuronal cells contribute to ALS pathogenesis. 
Understanding ALS: new therapeutic approaches
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Early Functional Deficit and Microglial Disturbances in a Mouse Model of Amyotrophic Lateral Sclerosis
Background Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by selective motoneurons degeneration. There is today no clear-cut pathogenesis sequence nor any
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State of the art and the dark side of amyotrophic lateral sclerosis.
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  • Biology
    World journal of biological chemistry
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Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice
Nonneuronal cells that do not express mutant SOD1 delay degeneration and significantly extend survival of mutant-expressing motor neurons.
Wild-type microglia extend survival in PU.1 knockout mice with familial amyotrophic lateral sclerosis
It is demonstrated that the expression of mSOD1G93A results in activated and neurotoxic microglia, and suggested that the lack of m SOD1 G93A expression inmicroglia may contribute to motoneuron protection, and confirmed the importance of microglio as a double-edged sword.
Restricted Expression of G86R Cu/Zn Superoxide Dismutase in Astrocytes Results in Astrocytosis But Does Not Cause Motoneuron Degeneration
Results indicate that 21q linked FALS is not a primary disorder of astrocytes, and that expression of mutant SOD1 restricted to astroCytes is not sufficient to cause motoneuron degeneration in vivo.
Absence of Tumor Necrosis Factor-α Does Not Affect Motor Neuron Disease Caused by Superoxide Dismutase 1 Mutations
Surprisingly, the absence of TNF-α did not affect the lifespan or the extent of motor neuron loss in SOD1 transgenic mice, indicating that T NF-α does not directly contribute to motor neuron degeneration caused by S OD1 mutations.
Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
Tight genetic linkage between FALS and a gene that encodes a cytosolic, Cu/Zn-binding superoxide dismutase (SOD1), a homodimeric metalloenzyme that catalyzes the dismutation of the toxic superoxide anion O–2 to O2 and H2O2 is reported.
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Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis
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Therapeutic effects of immunization with mutant superoxide dismutase in mice models of amyotrophic lateral sclerosis
It is proposed that immunization strategies should be considered as potential avenues for treatment of familial ALS caused by SOD1 mutations after emerging evidence for the existence of secretory pathways for superoxide dismutase (SOD1) mutants linked to amyotrophic lateral sclerosis (ALS).