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

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 muta...

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Autores principales:	Wei Liu, Zhi Chen, Yansen Ma, Xiaochun Wu, Yaping Jin, Steven Hou
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2013
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/93b77164b22b496cb31112d1b22b8d02
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spelling	oai:doaj.org-article:93b77164b22b496cb31112d1b22b8d022021-11-18T07:41:33ZGenetic characterization of the Drosophila birt-hogg-dubé syndrome gene.1932-620310.1371/journal.pone.0065869https://doaj.org/article/93b77164b22b496cb31112d1b22b8d022013-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/23799055/?tool=EBIhttps://doaj.org/toc/1932-6203Folliculin (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.Wei LiuZhi ChenYansen MaXiaochun WuYaping JinSteven HouPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 8, Iss 6, p e65869 (2013)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Wei Liu Zhi Chen Yansen Ma Xiaochun Wu Yaping Jin Steven Hou Genetic characterization of the Drosophila birt-hogg-dubé syndrome gene.
description	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.
format	article
author	Wei Liu Zhi Chen Yansen Ma Xiaochun Wu Yaping Jin Steven Hou
author_facet	Wei Liu Zhi Chen Yansen Ma Xiaochun Wu Yaping Jin Steven Hou
author_sort	Wei Liu
title	Genetic characterization of the Drosophila birt-hogg-dubé syndrome gene.
title_short	Genetic characterization of the Drosophila birt-hogg-dubé syndrome gene.
title_full	Genetic characterization of the Drosophila birt-hogg-dubé syndrome gene.
title_fullStr	Genetic characterization of the Drosophila birt-hogg-dubé syndrome gene.
title_full_unstemmed	Genetic characterization of the Drosophila birt-hogg-dubé syndrome gene.
title_sort	genetic characterization of the drosophila birt-hogg-dubé syndrome gene.
publisher	Public Library of Science (PLoS)
publishDate	2013
url	https://doaj.org/article/93b77164b22b496cb31112d1b22b8d02
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Genetic characterization of the Drosophila birt-hogg-dubé syndrome gene.

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