miR-206 Reduces the Severity of Motor Neuron Degeneration in the Facial Nuclei of the Brainstem in a Mouse Model of SMA

Spinal Muscular Atrophy (SMA) is a genetic disease that leads to the degeneration of motor neurons. This study focuses on the role of miR-206, a small RNA molecule, in SMA. The researchers found that miR-206 is initially upregulated in the brainstem of SMA mice during the early stages of the disease but is later downregulated as the disease progresses. To counteract this downregulation, they administered miR-206 to SMA mice. The administration of miR-206 resulted in a reduction in the severity of SMA, slowing down the progression of the disease, improving survival rates, and enhancing behavioral performance. They found that miR-206 reduces the expression of NCX2, which regulates calcium levels in cells.

• 1
  miR-206 is initially upregulated in the brainstem of SMA mice in the early stages of the disease, paralleling motor neuron degeneration, but downregulated in the late symptomatic phase.
• 2
  Intracerebroventricular injection of miR-206 in SMA pups reduces the severity of SMA pathology, slows disease progression, increases survival rate, and improves behavioral performance.
• 3
  Exogenous miR-206-induced upregulation caused a reduction of the predicted target sodium calcium exchanger isoform 2, NCX2, a regulator of intracellular calcium and sodium.