A new study reveals an unexpected mechanism underlying behavioral abnormalities in the neurodevelopmental disorder Williams syndrome. A deficit in myelination, resulting from the deletion of a Williams syndrome-associated gene in forebrain excitatory neurons, causes hypersociability by impairing action potential conduction. Accordingly, rescuing myelination or conduction normalizes this behavior.
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References
Pober, B. R. N. Engl. J. Med. 362, 239–252 (2010).
Barak, B. et al. Nat. Neurosci. https://doi.org/10.1038/s41593-019-0380-9 (2019).
Sakurai, T. et al. Autism Res. 4, 28–39 (2011).
Mei, F. et al. Nat. Med. 20, 954–960 (2014).
Liu, J. et al. J. Neurosci. 36, 957–962 (2016).
Hayes, K. C. CNS Drug Rev. 10, 295–316 (2004).
Zikopoulos, B. & Barbas, H. J. Neurosci. 30, 14595–14609 (2010).
Takahashi, N., Sakurai, T., Davis, K. L. & Buxbaum, J. D. Prog. Neurobiol. 93, 13–24 (2011).
Tomassy, G. S. et al. Science 344, 319–324 (2014).
Pajevic, S., Basser, P. J. & Fields, R. D. Neuroscience 276, 135–147 (2014).
Bostock, H., Sears, T. A. & Sherratt, R. M. J. Physiol. (Lond.) 313, 301–315 (1981).
Micheva, K. D. et al. eLife 5, e15784 (2016).
Hu, H., Gan, J. & Jonas, P. Science 345, 1255263 (2014).
Stedehouder, J. & Kushner, S. A. Mol. Psychiatry 22, 4–12 (2017).
Monje, M. Annu. Rev. Neurosci. 41, 61–76 (2018).
Acknowledgements
This work was supported by the NSERC (PGSD3-487560), the National Institutes of Health/National Institute of Neurological Disorders and Stroke (R01NS062796, R01NS097428, R01NS095889, R01NS088155), The Adelson Medical Research Foundation: ANDP (A130141), and the Rachleff Family Endowment.
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Osso, L.A., Chan, J.R. A surprising role for myelin in Williams syndrome. Nat Neurosci 22, 681–683 (2019). https://doi.org/10.1038/s41593-019-0368-5
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DOI: https://doi.org/10.1038/s41593-019-0368-5
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