Mitochondrial Dysfunction in Fragile-X Syndrome: Plugging the Leak May Save the Ship
- PMID: 33157013
- DOI: 10.1016/j.molcel.2020.10.002
Mitochondrial Dysfunction in Fragile-X Syndrome: Plugging the Leak May Save the Ship
Abstract
Recent work by Licznerski et al. suggests that mutant FMRP linked to Fragile-X syndrome elevates the inner mitochondrial membrane proton leak, leading to increased metabolism and changes in protein synthesis that trigger impaired synaptic maturation and autistic behaviors.
Copyright © 2020 Elsevier Inc. All rights reserved.
Comment on
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ATP Synthase c-Subunit Leak Causes Aberrant Cellular Metabolism in Fragile X Syndrome.Cell. 2020 Sep 3;182(5):1170-1185.e9. doi: 10.1016/j.cell.2020.07.008. Epub 2020 Aug 13. Cell. 2020. PMID: 32795412 Free PMC article.
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