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


ABSTRACT: 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.

SUBMITTER: Williams AH 

PROVIDER: S-EPMC2796560 | biostudies-literature | 2009 Dec

REPOSITORIES: biostudies-literature

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

Williams Andrew H AH   Valdez Gregorio G   Moresi Viviana V   Qi Xiaoxia X   McAnally John J   Elliott Jeffrey L JL   Bassel-Duby Rhonda R   Sanes Joshua R JR   Olson Eric N EN  

Science (New York, N.Y.) 20091201 5959


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. Mic  ...[more]

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