Nature Communications (Nov 2021)

Identification of disease-linked hyperactivating mutations in UBE3A through large-scale functional variant analysis

  • Kellan P. Weston,
  • Xiaoyi Gao,
  • Jinghan Zhao,
  • Kwang-Soo Kim,
  • Susan E. Maloney,
  • Jill Gotoff,
  • Sumit Parikh,
  • Yen-Chen Leu,
  • Kuen-Phon Wu,
  • Marwan Shinawi,
  • Joshua P. Steimel,
  • Joseph S. Harrison,
  • Jason J. Yi

DOI
https://doi.org/10.1038/s41467-021-27156-0
Journal volume & issue
Vol. 12, no. 1
pp. 1 – 15

Abstract

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UBE3A gene dysregulation is associated with neurodevelopmental disorders, but predicting the function of UBE3A variants remains difficult. The authors use a high-throughput assay to categorize variants by functional activity, and show that UBE3A hyperactivity increases the risk of neurodevelopmental disease.