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

Science (New York, N.Y.) | Dec 11, 2009

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 RIS Download

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

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Associated grants

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

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