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- W3010017013 abstract "FLCN was identified as the gene responsible for Birt-Hogg-Dubé (BHD) syndrome, a hereditary syndrome associated with the appearance of familiar renal oncocytomas. Most mutations affecting FLCN result in the truncation of the protein, and therefore loss of its associated functions, as typical for a tumour suppressor. FLCN encodes the protein folliculin (FLCN), which is involved in numerous biological processes; mutations affecting this protein thus lead to different phenotypes depending on the cellular context. FLCN forms complexes with two large interacting proteins, FNIP1 and FNIP2. Structural studies have shown that both FLCN and FNIPs contain longin and differentially expressed in normal versus neoplastic cells (DENN) domains, typically involved in the regulation of small GTPases. Accordingly, functional studies show that FLCN regulates both the Rag and the Rab GTPases depending on nutrient availability, which are respectively involved in the mTORC1 pathway and lysosomal positioning. Although recent structural studies shed light on the precise mechanism by which FLCN regulates the Rag GTPases, which in turn regulate mTORC1, how FLCN regulates membrane trafficking through the Rab GTPases or the significance of the intriguing FLCN-FNIP-AMPK complex formation are questions that still remain unanswered. We discuss the recent progress in our understanding of FLCN regulation of both growth signalling and lysosomal positioning, as well as future approaches to establish detailed mechanisms to explain the disparate phenotypes caused by the loss of FLCN function and the development of BHD-associated and other tumours." @default.
- W3010017013 created "2020-03-13" @default.
- W3010017013 creator A5032946129 @default.
- W3010017013 creator A5034067020 @default.
- W3010017013 date "2020-03-03" @default.
- W3010017013 modified "2023-09-28" @default.
- W3010017013 title "Nutrient Signaling and Lysosome Positioning Crosstalk Through a Multifunctional Protein, Folliculin" @default.
- W3010017013 cites W1205458192 @default.
- W3010017013 cites W1534985842 @default.
- W3010017013 cites W1545683217 @default.
- W3010017013 cites W1821767256 @default.
- W3010017013 cites W1969989556 @default.
- W3010017013 cites W1972762474 @default.
- W3010017013 cites W1973201313 @default.
- W3010017013 cites W1975200363 @default.
- W3010017013 cites W1977534471 @default.
- W3010017013 cites W1978260512 @default.
- W3010017013 cites W1979752285 @default.
- W3010017013 cites W1981648940 @default.
- W3010017013 cites W1983834588 @default.
- W3010017013 cites W1985370440 @default.
- W3010017013 cites W1985754630 @default.
- W3010017013 cites W1987670956 @default.
- W3010017013 cites W1990278643 @default.
- W3010017013 cites W1990960113 @default.
- W3010017013 cites W1992987381 @default.
- W3010017013 cites W1995455069 @default.
- W3010017013 cites W1997836389 @default.
- W3010017013 cites W2000678720 @default.
- W3010017013 cites W2002717894 @default.
- W3010017013 cites W2003010123 @default.
- W3010017013 cites W2004582874 @default.
- W3010017013 cites W2006559495 @default.
- W3010017013 cites W2008239768 @default.
- W3010017013 cites W2011282245 @default.
- W3010017013 cites W2011437516 @default.
- W3010017013 cites W2013381376 @default.
- W3010017013 cites W2016894006 @default.
- W3010017013 cites W2017058729 @default.
- W3010017013 cites W2020257412 @default.
- W3010017013 cites W2023012828 @default.
- W3010017013 cites W2023819606 @default.
- W3010017013 cites W2024069519 @default.
- W3010017013 cites W2025176940 @default.
- W3010017013 cites W2027785297 @default.
- W3010017013 cites W2031918765 @default.
- W3010017013 cites W2032848771 @default.
- W3010017013 cites W2038982391 @default.
- W3010017013 cites W2042710439 @default.
- W3010017013 cites W2043287555 @default.
- W3010017013 cites W2044874695 @default.
- W3010017013 cites W2046447620 @default.
- W3010017013 cites W2051266786 @default.
- W3010017013 cites W2057106513 @default.
- W3010017013 cites W2058834594 @default.
- W3010017013 cites W2059185581 @default.
- W3010017013 cites W2063013559 @default.
- W3010017013 cites W2065887259 @default.
- W3010017013 cites W2070910260 @default.
- W3010017013 cites W2077091529 @default.
- W3010017013 cites W2077499130 @default.
- W3010017013 cites W2077858999 @default.
- W3010017013 cites W2078184757 @default.
- W3010017013 cites W2078765043 @default.
- W3010017013 cites W2080100938 @default.
- W3010017013 cites W2080481362 @default.
- W3010017013 cites W2081384263 @default.
- W3010017013 cites W2081920342 @default.
- W3010017013 cites W2083087851 @default.
- W3010017013 cites W2083655799 @default.
- W3010017013 cites W2084712051 @default.
- W3010017013 cites W2086053245 @default.
- W3010017013 cites W2086327073 @default.
- W3010017013 cites W2086698861 @default.
- W3010017013 cites W2087812126 @default.
- W3010017013 cites W2089632625 @default.
- W3010017013 cites W2092122970 @default.
- W3010017013 cites W2092636253 @default.
- W3010017013 cites W2094517081 @default.
- W3010017013 cites W2096308078 @default.
- W3010017013 cites W2096750932 @default.
- W3010017013 cites W2099795938 @default.
- W3010017013 cites W2100127220 @default.
- W3010017013 cites W2100902550 @default.
- W3010017013 cites W2102891343 @default.
- W3010017013 cites W2103516315 @default.
- W3010017013 cites W2104316079 @default.
- W3010017013 cites W2114859236 @default.
- W3010017013 cites W2119884717 @default.
- W3010017013 cites W2119914706 @default.
- W3010017013 cites W2121211842 @default.
- W3010017013 cites W2122568309 @default.
- W3010017013 cites W2127063807 @default.
- W3010017013 cites W2127088485 @default.
- W3010017013 cites W2130475925 @default.
- W3010017013 cites W2131856428 @default.
- W3010017013 cites W2132463881 @default.
- W3010017013 cites W2137315893 @default.
- W3010017013 cites W2139948451 @default.
- W3010017013 cites W2140801892 @default.