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MicroRNA-206 delays ALS progression and promotes regeneration of neuromuscular synapses in mice.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by loss of motor neurons, denervation of target muscles, muscle atrophy, and paralysis. Understanding ALS pathogenesis may require a fuller understanding of the bidirectional signaling between motor neurons and skeletal muscle fibers at neuromuscular synapses. Here, we show that a key regulator of this signaling is miR-206, a skeletal muscle-specific microRNA that is dramatically induced in a mouse model of ALS. Mice that are genetically deficient in miR-206 form normal neuromuscular synapses during development, but deficiency of miR-206 in the ALS mouse model accelerates disease progression. miR-206 is required for efficient regeneration of neuromuscular synapses after acute nerve injury, which probably accounts for its salutary effects in ALS. miR-206 mediates these effects at least in part through histone deacetylase 4 and fibroblast growth factor signaling pathways. Thus, miR-206 slows ALS progression by sensing motor neuron injury and promoting the compensatory regeneration of neuromuscular synapses.

Pubmed ID: 20007902


  • Williams AH
  • Valdez G
  • Moresi V
  • Qi X
  • McAnally J
  • Elliott JL
  • Bassel-Duby R
  • Sanes JR
  • Olson EN


Science (New York, N.Y.)

Publication Data

December 11, 2009

Associated Grants

  • Agency: NINDS NIH HHS, Id: 1F32NS061464-01A1
  • Agency: NHLBI NIH HHS, Id: R01 HL093039
  • Agency: NHLBI NIH HHS, Id: R01 HL093039-01A1
  • Agency: NHLBI NIH HHS, Id: T32HL007360
  • Agency: NCI NIH HHS, Id: U24 CA126608

Mesh Terms

  • Amyotrophic Lateral Sclerosis
  • Animals
  • Axons
  • Carrier Proteins
  • Disease Models, Animal
  • Disease Progression
  • Fibroblast Growth Factors
  • Histone Deacetylases
  • Mice
  • Mice, Transgenic
  • MicroRNAs
  • Motor Neurons
  • Muscle Denervation
  • Muscle, Skeletal
  • MyoD Protein
  • Myogenin
  • Nerve Regeneration
  • Neuromuscular Junction
  • RNA Interference
  • Signal Transduction
  • Transcriptional Activation
  • Up-Regulation