Nature Communications (Oct 2020)

Intellectual disability-associated factor Zbtb11 cooperates with NRF-2/GABP to control mitochondrial function

  • Brooke C. Wilson,
  • Lena Boehme,
  • Ambra Annibali,
  • Alan Hodgkinson,
  • Thomas S. Carroll,
  • Rebecca J. Oakey,
  • Vlad C. Seitan

DOI
https://doi.org/10.1038/s41467-020-19205-x
Journal volume & issue
Vol. 11, no. 1
pp. 1 – 20

Abstract

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ZBTB11 mutations have been identified in patients with intellectual disability and morphological brain and neuromuscular defects, although the etiology was unknown. Here, the authors demonstrate that ZBTB11 regulates mitochondrial function by facilitating NRF-2-mediated activation of complex I and mitoribosome genes.