The function of astrocytes is altered in Duchenne muscular dystrophy (DMD), according to a new study published in Glia.
Jenny Lange, PhD, and the coauthors of the study suggest that defective astrocyte responses may contribute to neural impairment in patients with DMD. The data also suggest that targeting astrocytes could be a viable therapeutic option for these patients.
A third of patients with DMD have cognitive deficits in addition to muscle degeneration and scarring. Although it is not clear how defective dystrophin affects the brain, it is thought that the deficiency of the protein may affect the function of glial cells.
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To test this hypothesis, the authors investigated the expression of human dystrophin isoforms in human astrocytes from control and induced pluripotent stem cells in patients with DMD.
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They found that in control cells, short isoforms of the dystrophin protein were upregulated with development. Moreover, their expression levels changed differently upon neuronal and astrocytic differentiation.
When the researchers looked at DMD astrocytes, they found that the morphology of all of them was altered, and they all showed proliferative activity. However, their morphology did not change in response to inflammation, and their numbers were significantly lower than stimulated healthy astrocytes.
Finally, DMD astrocytes were more sensitive to oxidative damage compared to controls, as they had increased cell death.
“Together, our DMD model provides evidence for altered astrocyte function in DMD,” the researchers wrote. “Therefore, to ameliorate neural impairments in DMD patients, therapies should also target these cells.“
Astrocytes are cells that support the central nervous system and play a critical role in repair, following injury or infection.
Reference
Lange J, Gillham O, Alkharji R, et al. Dystrophin deficiency affects human astrocyte properties and response to damage. Glia. Published online November 13, 2021. doi:10.1002/glia.24116
