PLoS ONE (Jan 2013)

Genetic characterization of the Drosophila birt-hogg-dubé syndrome gene.

  • Wei Liu,
  • Zhi Chen,
  • Yansen Ma,
  • Xiaochun Wu,
  • Yaping Jin,
  • Steven Hou

DOI
https://doi.org/10.1371/journal.pone.0065869
Journal volume & issue
Vol. 8, no. 6
p. e65869

Abstract

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Folliculin (FLCN) is a conserved tumor suppressor gene whose loss is associated with the human Birt-Hogg-Dubé (BHD) syndrome. However, its molecular functions remain largely unknown. In this work, we generated a Drosophila BHD model through genomic deletion of the FLCN gene (DBHD(-) ). The DBHD mutant larvae grew slowly and stopped development before pupation, displaying various characteristics of malnutrition. We found the growth delay was sensitive to the nutrient supplies. It became more severe upon restrictions of the dietary yeast; while high levels of yeast significantly restored the normal growth, but not viability. We further demonstrated that leucine was able to substitute for yeast to provide similar rescues. Moreover, the human FLCN could partially rescue the DBHD(-) phenotypes, indicating the two genes are involved in certain common mechanisms. Our work provides a new animal model of the BHD syndrome and suggests that modulation of the local nutrient condition might be a potential treatment of the BHD lesions.