Редактирование: FLCN

Folliculin (BHD skin lesion fibrofolliculoma protein) (Birt-Hogg-Dube syndrome protein) [BHD]

==Publications==

{{medline-entry
|title=Loss of the Birt-Hogg-Dubé gene product folliculin induces longevity in a hypoxia-inducible factor-dependent manner.
|pubmed-url=https://pubmed.ncbi.nlm.nih.gov/23566034
|abstract=Signaling through the hypoxia-inducible factor hif-1 controls longevity, metabolism, and stress resistance in Caenorhabditis elegans. Hypoxia-inducible factor (HIF) protein levels are regulated through an evolutionarily conserved ubiquitin ligase complex. Mutations in the [[VHL]] gene, encoding a core component of this complex, cause a multitumor syndrome and renal cell carcinoma in humans. In the nematode, deficiency in vhl-1 promotes longevity mediated through HIF-1 stabilization. However, this longevity assurance pathway is not yet understood. Here, we identify folliculin ([[FLCN]]) as a novel interactor of the hif-1/vhl-1 longevity pathway. [[FLCN]] mutations cause Birt-Hogg-Dubé syndrome in humans, another tumor syndrome with renal tumorigenesis reminiscent of the [[VHL]] disease. Loss of the C. elegans ortholog of [[FLCN]] F22D3.2 significantly increased lifespan and enhanced stress resistance in a hif-1-dependent manner. F22D3.2, vhl-1, and hif-1 control longevity by a mechanism distinct from insulin-like signaling. Daf-16 deficiency did not abrogate the increase in lifespan mediated by flcn-1. These findings define [[FLCN]] as a player in HIF-dependent longevity signaling and connect organismal aging, stress resistance, and regulation of longevity with the formation of renal cell carcinoma.
|mesh-terms=* Animals
* Birt-Hogg-Dube Syndrome
* Caenorhabditis elegans
* Caenorhabditis elegans Proteins
* Cullin Proteins
* Forkhead Transcription Factors
* Gene Expression Regulation, Developmental
* Humans
* Longevity
* Mutation
* Protein Stability
* Proto-Oncogene Proteins
* RNA, Messenger
* Sequence Homology, Amino Acid
* Transcription Factors
* Tumor Suppressor Proteins
|keywords=* Birt-Hogg-Dubé
* C. elegans
* Vhl
* folliculin
* hif
* longevity
|full-text-url=https://sci-hub.do/10.1111/acel.12081
}}