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Brainy Cells and Brawny Cells, Both Built From Similar Stuff

  • Published28 Jan 2020
  • Author Charlie Wood
  • Source BrainFacts/SfN

A mouse nerve threads its way through a sea of muscle cells. Although nerve cells bring the brains while muscle cells bring the brawn, the two unlikely bedfellows share similar building materials. The red silhouettes above highlight a protein called dystrophin, which helps keep muscle cells firm and strong. The green outlines represent “dystrophin-like protein” — its official name — which does something similar for the fatty myelin coverings that surround nerve cells.

Researchers have long known that genetic errors can wreck dystrophin, leading to disorders of weak muscles, such as muscular dystrophy. In recent years, neuroscientists have recognized that the same thing can happen to nerves. A breakdown in the production of dystrophin-like protein has been linked to damaged nerve casings, and a type of genetic disease known as Charcot–Marie–Tooth disease where a failure to efficiently transmit information leaves hands and feet numb.

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BrainFacts/SfN

Brennan, K. M., Bai, Y., Pisciotta, C., Wang, S., Feely, S. M. E., Hoegger, M., … Shy, M. E. (2015). Absence of Dystrophin Related Protein-2 disrupts Cajal bands in a patient with Charcot-Marie-Tooth disease. Neuromuscular Disorders: NMD, 25(10), 786–793. doi: 10.1016/j.nmd.2015.07.001

Gao, Q. Q., & McNally, E. M. (2015). The Dystrophin Complex: Structure, Function, and Implications for Therapy. Comprehensive Physiology, 5(3), 1223–1239. doi: 10.1002/cphy.c140048

Sherman, D. L., Fabrizi, C., Gillespie, C. S., & Brophy, P. J. (2001). Specific Disruption of a Schwann Cell Dystrophin-Related Protein Complex in a Demyelinating Neuropathy. Neuron, 30(3), 677–687. doi: 10.1016/S0896-6273(01)00327-0

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