ALS: A Disease of Motor Neurons and Their Nonneuronal Neighbors

  title={ALS: A Disease of Motor Neurons and Their Nonneuronal Neighbors},
  author={S. Boill{\'e}e and C. Velde and D. Cleveland},
  • S. Boillée, C. Velde, D. Cleveland
  • Published 2006
  • Medicine, Biology
  • Neuron
  • Amyotrophic lateral sclerosis is a late-onset progressive neurodegenerative disease affecting motor neurons. The etiology of most ALS cases remains unknown, but 2% of instances are due to mutations in Cu/Zn superoxide dismutase (SOD1). Since sporadic and familial ALS affects the same neurons with similar pathology, it is hoped that therapies effective in mutant SOD1 models will translate to sporadic ALS. Mutant SOD1 induces non-cell-autonomous motor neuron killing by an unknown gain of toxicity… CONTINUE READING
    Current hypotheses for the underlying biology of amyotrophic lateral sclerosis
    • 612
    • Highly Influenced
    • PDF
    Rodent Models of Amyotrophic Lateral Sclerosis
    • 105
    The “Dying-Back” Phenomenon of Motor Neurons in ALS
    • 235
    • PDF


    Publications referenced by this paper.
    Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia
    • 1,354
    • PDF
    ALS-Linked SOD1 Mutant G85R Mediates Damage to Astrocytes and Promotes Rapidly Progressive Disease with SOD1-Containing Inclusions
    • 1,235
    • Highly Influential
    Massive Mitochondrial Degeneration in Motor Neurons Triggers the Onset of Amyotrophic Lateral Sclerosis in Mice Expressing a Mutant SOD1
    • 675
    • Highly Influential
    • PDF
    Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice
    • 1,003
    • PDF
    Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man
    • 1,037
    • PDF
    Aggregation of Mutant Cu/Zn Superoxide Dismutase Proteins in a Culture Model of ALS
    • 358
    • PDF
    Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
    • 6,014
    • Highly Influential