AI Article Synopsis
- Duchenne muscular dystrophy (DMD) not only impacts muscles but also leads to memory and behavioral issues due to effects on the brain.
- Researchers used high-resolution MRI on mouse models to study how varying levels of dystrophin (none, full, or low) influenced brain structure.
- Findings indicate that while overall brain volume and skull shape were mostly unaffected by dystrophin levels, specific brain structures showed volume differences, echoing patterns observed in humans with DMD.
Article Abstract
Duchenne muscular dystrophy (DMD) affects besides muscle also the brain, resulting in memory and behavioral problems. The consequences of dystrophinopathy on gross macroscopic alterations are unclear. To elucidate the effect of full-length dystrophin expression on brain morphology, we used high-resolution post-mortem MRI in mouse models that either express 0% (mdx), 100% (BL10) or a low amount of full-length dystrophin (mdx-XistΔhs). While absence or low amounts of full-length dystrophin did not significantly affect whole brain volume and skull morphology, we found differences in volume of individual brain structures. The results are in line with observations in humans, where whole brain volume was found to be reduced only in patients lacking both full-length dystrophin and the shorter isoform Dp140.
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Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5877835 | PMC |
| http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0194636 | PLOS |
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