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Select item 70087051.

Cryo-EM structure of the human FLCN-FNIP2-Rag-Ragulator complex

Kuang Shen, Kacper B. Rogala, Hui-Ting Chou, Rick K. Huang, Zhiheng Yu, David M. Sabatini

Cell. Author manuscript; available in PMC 2020 Nov 27.

Published in final edited form as: Cell. 2019 Nov 27; 179(6): 1319–1329.e8. Published online 2019 Nov 6. doi: 10.1016/j.cell.2019.10.036

PMCID:: PMC7008705

Abstract Article PDF–2.3M

Select item 69458162.

Structural Mechanism of a Rag GTPase Activation Checkpoint by the Lysosomal Folliculin Complex

Rosalie E. Lawrence, Simon A. Fromm, Yangxue Fu, Adam L. Yokom, Do Jin Kim, Ashley M. Thelen, Lindsey N. Young, Chun-Yan Lim, Avi J. Samelson, James H. Hurley, Roberto Zoncu

Science. Author manuscript; available in PMC 2020 May 22.

Published in final edited form as: Science. 2019 Nov 22; 366(6468): 971–977. Published online 2019 Oct 31. doi: 10.1126/science.aax0364

PMCID:: PMC6945816

Abstract Article PDF–2.5M

Select item 60809353.

GATOR1-dependent recruitment of FLCN–FNIP to lysosomes coordinates Rag GTPase heterodimer nucleotide status in response to amino acids

Jin Meng, Shawn M. Ferguson

J Cell Biol. 2018 Aug 6; 217(8): 2765–2776. doi: 10.1083/jcb.201712177

PMCID:: PMC6080935

Abstract Article PDF–2.6M

Select item 51596694.

The tumor suppressor FLCN mediates an alternate mTOR pathway to regulate browning of adipose tissue

Shogo Wada, Michael Neinast, Cholsoon Jang, Yasir H. Ibrahim, Gina Lee, Apoorva Babu, Jian Li, Atsushi Hoshino, Glenn C. Rowe, James Rhee, José A. Martina, Rosa Puertollano, John Blenis, Michael Morley, Joseph A. Baur, Patrick Seale, Zoltan Arany

Genes Dev. 2016 Nov 15; 30(22): 2551–2564. doi: 10.1101/gad.287953.116

PMCID:: PMC5159669

Abstract Article PDF–1.6M

Select item 50673005.

Amino Acid Sensing by mTORC1: Intracellular Transporters Mark the Spot

Deborah C.I. Goberdhan, Clive Wilson, Adrian L. Harris

Cell Metab. 2016 Apr 12; 23(4): 580–589. doi: 10.1016/j.cmet.2016.03.013

PMCID:: PMC5067300

Abstract Article PDF–2.2M

Select item 48938186.

Folliculin directs the formation of a Rab34–RILP complex to control the nutrient‐dependent dynamic distribution of lysosomes

Georgina P Starling, Yan Y Yip, Anneri Sanger, Penny E Morton, Emily R Eden, Mark P Dodding

EMBO Rep. 2016 Jun; 17(6): 823–841. Published online 2016 Apr 13. doi: 10.15252/embr.201541382

PMCID:: PMC4893818

Abstract Article PDF–4.7M

Select item 48637357.

Chronic AMPK activation via loss of FLCN induces functional beige adipose tissue through PGC-1α/ERRα

Ming Yan, Étienne Audet-Walsh, Sanaz Manteghi, Catherine Rosa Dufour, Benjamin Walker, Masaya Baba, Julie St-Pierre, Vincent Giguère, Arnim Pause

Genes Dev. 2016 May 1; 30(9): 1034–1046. doi: 10.1101/gad.281410.116

PMCID:: PMC4863735

Abstract Article PDF–642K

Select item 43863368.

Folliculin-interacting proteins Fnip1 and Fnip2 play critical roles in kidney tumor suppression in cooperation with Flcn

Hisashi Hasumi, Masaya Baba, Yukiko Hasumi, Martin Lang, Ying Huang, HyoungBin F. Oh, Masayuki Matsuo, Maria J. Merino, Masahiro Yao, Yusuke Ito, Mitsuko Furuya, Yasuhiro Iribe, Tatsuhiko Kodama, Eileen Southon, Lino Tessarollo, Kunio Nagashima, Diana C. Haines, W. Marston Linehan, Laura S. Schmidt

Proc Natl Acad Sci U S A. 2015 Mar 31; 112(13): E1624–E1631. Published online 2015 Mar 16. doi: 10.1073/pnas.1419502112

PMCID:: PMC4386336

Abstract Article PDF–1.9M

Select item 41983609.

FLCN, a novel autophagy component, interacts with GABARAP and is regulated by ULK1 phosphorylation

Elaine A Dunlop, Sara Seifan, Tijs Claessens, Christian Behrends, Miriam AF Kamps, Ewelina Rozycka, Alain J Kemp, Ravi K Nookala, John Blenis, Barry J Coull, James T Murray, Maurice AM van Steensel, Simon Wilkinson, Andrew R Tee

Autophagy. 2014 Oct 1; 10(10): 1749–1760. Published online 2014 Jul 22. doi: 10.4161/auto.29640

PMCID:: PMC4198360

Abstract Article PDF–5.2M

Select item 386781710.

The Folliculin tumor suppressor is a GAP for RagC/D GTPases that signal amino acid levels to mTORC1

Zhi-Yang Tsun, Liron Bar-Peled, Lynne Chantranupong, Roberto Zoncu, Tim Wang, Choah Kim, Eric Spooner, David M. Sabatini

Mol Cell. Author manuscript; available in PMC 2014 Nov 21.

Published in final edited form as: Mol Cell. 2013 Nov 21; 52(4): 10.1016/j.molcel.2013.09.016. Published online 2013 Oct 3. doi: 10.1016/j.molcel.2013.09.016

PMCID:: PMC3867817

Abstract Article PDF–3.2M

Select item 379269511.

Birt–Hogg–Dubé syndrome is a novel ciliopathy

Monique N.H. Luijten, Sander G. Basten, Tijs Claessens, Marigje Vernooij, Claire L. Scott, Renske Janssen, Jennifer A. Easton, Miriam A.F. Kamps, Maaike Vreeburg, Jos L.V. Broers, Michel van Geel, Fred H. Menko, Richard P. Harbottle, Ravi K. Nookala, Andrew R. Tee, Stephen C. Land, Rachel H. Giles, Barry J. Coull, Maurice A.M. van Steensel

Hum Mol Genet. 2013 Nov 1; 22(21): 4383–4397. Published online 2013 Jun 19. doi: 10.1093/hmg/ddt288

PMCID:: PMC3792695

Abstract Article PDF–1.3M

Select item 378738212.

Recruitment of folliculin to lysosomes supports the amino acid–dependent activation of Rag GTPases

Constance S. Petit, Agnes Roczniak-Ferguson, Shawn M. Ferguson

J Cell Biol. 2013 Sep 30; 202(7): 1107–1122. doi: 10.1083/jcb.201307084

PMCID:: PMC3787382

Abstract Article PDF–4.1M

Select item 375551113.

Folliculin interacts with p0071 (plakophilin-4) and deficiency is associated with disordered RhoA signalling, epithelial polarization and cytokinesis

Michael S. Nahorski, Laurence Seabra, Ania Straatman-Iwanowska, Aileen Wingenfeld, Anne Reiman, Xiaohong Lu, Jeff A. Klomp, Bin T. Teh, Mechthild Hatzfeld, Paul Gissen, Eamonn R. Maher

Hum Mol Genet. 2012 Dec 15; 21(24): 5268–5279. Published online 2012 Sep 10. doi: 10.1093/hmg/dds378

PMCID:: PMC3755511

Abstract Article PDF–2.1M

Select item 350219614.

Regulation of Mitochondrial Oxidative Metabolism by Tumor Suppressor FLCN

Hisashi Hasumi, Masaya Baba, Yukiko Hasumi, Ying Huang, Hyoungbin Oh, Robert M. Hughes, Mara E. Klein, Shoichi Takikita, Kunio Nagashima, Laura S. Schmidt, W. Marston Linehan

J Natl Cancer Inst. 2012 Nov 21; 104(22): 1750–1764. Published online 2012 Nov 20. doi: 10.1093/jnci/djs418

PMCID:: PMC3502196

Abstract Article PDF–969K

Select item 341872015.

The folliculin-FNIP1 pathway deleted in human Birt-Hogg-Dubé syndrome is required for murine B-cell development

Masaya Baba, Jonathan R. Keller, Hong-Wei Sun, Wolfgang Resch, Stefan Kuchen, Hyung Chan Suh, Hisashi Hasumi, Yukiko Hasumi, Kyong-Rim Kieffer-Kwon, Carme Gallego Gonzalez, Robert M. Hughes, Mara E. Klein, Hyoungbin F. Oh, Paul Bible, Eileen Southon, Lino Tessarollo, Laura S. Schmidt, W. Marston Linehan, Rafael Casellas

Blood. 2012 Aug 9; 120(6): 1254–1261. Prepublished online 2012 Jun 18. doi: 10.1182/blood-2012-02-410407

PMCID:: PMC3418720

Abstract Article

Select item 317805916.

Phylogenetic-based propagation of functional annotations within the Gene Ontology consortium

Pascale Gaudet, Michael S. Livstone, Suzanna E. Lewis, Paul D. Thomas

Brief Bioinform. 2011 Sep; 12(5): 449–462. Published online 2011 Aug 27. doi: 10.1093/bib/bbr042

PMCID:: PMC3178059

Abstract Article PDF–1.0M

Select item 310927017.

Loss of the Birt–Hogg–Dubé tumor suppressor results in apoptotic resistance due to aberrant TGFβ-mediated transcription

T P Cash, J J Gruber, T R Hartman, E P Henske, M C Simon

Oncogene. 2011 Jun 2; 30(22): 2534–2546. Published online 2011 Jan 24. doi: 10.1038/onc.2010.628

PMCID:: PMC3109270

Abstract Article PDF–921K

Select item 301211718.

Inactivation of the FLCN Tumor Suppressor Gene Induces TFE3 Transcriptional Activity by Increasing Its Nuclear Localization

Seung-Beom Hong, HyoungBin Oh, Vladimir A. Valera, Masaya Baba, Laura S. Schmidt, W. Marston Linehan

PLoS One. 2010; 5(12): e15793. Published online 2010 Dec 29. doi: 10.1371/journal.pone.0015793

PMCID:: PMC3012117

Abstract Article PDF–3.7M

Select item 290732919.

Tumor suppressor FLCN inhibits tumorigenesis of a FLCN-null renal cancer cell line and regulates expression of key molecules in TGF-β signaling

Seung-Beom Hong, HyoungBin Oh, Vladimir A Valera, Jaime Stull, Duy-Tan Ngo, Masaya Baba, Maria J Merino, W Marston Linehan, Laura S Schmidt

Mol Cancer. 2010; 9: 160. Published online 2010 Jun 23. doi: 10.1186/1476-4598-9-160

PMCID:: PMC2907329

Abstract Article PDF–4.5M

Select item 276592520.

Homozygous loss of BHD causes early embryonic lethality and kidney tumor development with activation of mTORC1 and mTORC2

Yukiko Hasumi, Masaya Baba, Rieko Ajima, Hisashi Hasumi, Vladimir A. Valera, Mara E. Klein, Diana C. Haines, Maria J. Merino, Seung-Beom Hong, Terry P. Yamaguchi, Laura S. Schmidt, W. Marston Linehan

Proc Natl Acad Sci U S A. 2009 Nov 3; 106(44): 18722–18727. Published online 2009 Oct 22. doi: 10.1073/pnas.0908853106