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Stem Cells Get Motoring!

Motor neuron diseases encompass a range of neurological disorders that affect the muscles and movement of the body. These disorders are caused by the progressive degeneration of motor neurons, often leading to paralysis and ultimately death in patients. Can a stem cell-based strategy provide replacement cells?

Researchers at the Institute for Stem Cell Therapy and Exploration of Monogenic Diseases (I-Stem – Inserm/AFM/UEVE), in collaboration with CNRS and Paris Descartes University now seem to have developed the optimal recipe for making different types of motor neurons from human pluripotent stem cells (hPSCs). Differentiation is usually a long, drawn out inefficient process, but this new strategy can produce a highly homogenous population of cells in half the normal time; a remarkable 14 days.

The scalable, automatic hPSC differentiation approach taken by the group allowed the systematic testing of small molecule combinations, ultimately revealing an important role for Wnt signaling, alongside contributions by FGF, retinoic acid and Hedgehog signaling. These findings permitted the generation of functional spinal and cranial motor neurons as well as spinal interneurons and sensory neurons, each produced by tweaking the intensity and timing of each signaling pathway.

Why are these cells useful? They can be used to:

  • Produce and study the basic biology of motor neurons
  • Analyze motor neural degeneration in disease models to provide therapeutic information
  • Treat paralytic diseases such as infantile spinal muscular amyotrophy or amyotrophic lateral sclerosis
  • Test drugs which may preserve endogenous motor neuron function

These findings hold much promise in accelerating the pace of clinical advances in this field, so don’t let this paper speed by, see this incredible study here.