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NEURODEVELOPMENTAL DISORDERS

A surprising role for myelin in Williams syndrome

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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Fig. 1: A new model of Williams syndrome.

References

  1. Pober, B. R. N. Engl. J. Med. 362, 239–252 (2010).

    Article  CAS  Google Scholar 

  2. Barak, B. et al. Nat. Neurosci. https://doi.org/10.1038/s41593-019-0380-9 (2019).

    Article  Google Scholar 

  3. Sakurai, T. et al. Autism Res. 4, 28–39 (2011).

    Article  Google Scholar 

  4. Mei, F. et al. Nat. Med. 20, 954–960 (2014).

    Article  CAS  Google Scholar 

  5. Liu, J. et al. J. Neurosci. 36, 957–962 (2016).

    Article  CAS  Google Scholar 

  6. Hayes, K. C. CNS Drug Rev. 10, 295–316 (2004).

    Article  CAS  Google Scholar 

  7. Zikopoulos, B. & Barbas, H. J. Neurosci. 30, 14595–14609 (2010).

    Article  CAS  Google Scholar 

  8. Takahashi, N., Sakurai, T., Davis, K. L. & Buxbaum, J. D. Prog. Neurobiol. 93, 13–24 (2011).

    Article  CAS  Google Scholar 

  9. Tomassy, G. S. et al. Science 344, 319–324 (2014).

    Article  CAS  Google Scholar 

  10. Pajevic, S., Basser, P. J. & Fields, R. D. Neuroscience 276, 135–147 (2014).

    Article  CAS  Google Scholar 

  11. Bostock, H., Sears, T. A. & Sherratt, R. M. J. Physiol. (Lond.) 313, 301–315 (1981).

    Article  CAS  Google Scholar 

  12. Micheva, K. D. et al. eLife 5, e15784 (2016).

    Article  Google Scholar 

  13. Hu, H., Gan, J. & Jonas, P. Science 345, 1255263 (2014).

    Article  Google Scholar 

  14. Stedehouder, J. & Kushner, S. A. Mol. Psychiatry 22, 4–12 (2017).

    Article  CAS  Google Scholar 

  15. Monje, M. Annu. Rev. Neurosci. 41, 61–76 (2018).

    Article  CAS  Google Scholar 

Download references

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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Correspondence to Jonah R. Chan.

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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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