The unconditioned fear response in vertebrates deficient in dystrophin

S. Gharibi, C. Vaillend, A. Lindsay
Progress in Neurobiology, Volume 235, 2024, 102590, ISSN 0301-0082

Lay Summary

The review paper titled “The Unconditioned Fear Response in Vertebrates Deficient in Dystrophin,” published in Progress in Neurobiology, describes how the absence of dystrophin, a protein crucial for muscle function, also affects stress reactivity. This is important for understanding Duchenne muscular dystrophy (DMD), a genetic disorder where dystrophin is missing.

Dystrophin is not only important in muscle tissue but also plays a significant role in the brain. Its deficiency can lead to cognitive and behavioral abnormalities. The review highlights that mice or patients lacking dystrophin exhibit hypersensitivity to stressors and enhanced anxiety. This suggest their brains process fear and stress differently from people without DMD.

The paper explains that these changes are in part due to problems in some of the brain’s chemicals used for cells to communicate with each other, particularly GABA, which controls fear and anxiety. Key brain areas involved by the lack of dystrophin include the amygdala and hypothalamus. A critical interplay between brain and other organs are also highlighted, pointing to dysfunctions of the autonomic nervous system and regulation of stress hormone and blood pressure.

In summary, the lack of dystrophin affects not just muscles but also the brain and emotions. Understanding this can help develop treatments to improve both physical and emotional health in people with DMD.