Deficiency of FLCN in mouse kidney led to development of polycystic kidneys and renal neoplasia
about
Folliculin interacts with p0071 (plakophilin-4) and deficiency is associated with disordered RhoA signalling, epithelial polarization and cytokinesisCrystal structure of folliculin reveals a hidDENN function in genetically inherited renal cancerUBE2QL1 is disrupted by a constitutional translocation associated with renal tumor predisposition and is a novel candidate renal tumor suppressor geneInactivation of the FLCN tumor suppressor gene induces TFE3 transcriptional activity by increasing its nuclear localizationTumor suppressor FLCN inhibits tumorigenesis of a FLCN-null renal cancer cell line and regulates expression of key molecules in TGF-beta signalingGenetic and Chromosomal Aberrations and Their Clinical Significance in Renal NeoplasmsAccessory proteins for heterotrimeric G-proteins in the kidneyAmino acid management in cancerActivators of G protein signaling in the kidneyExpression and knockdown of zebrafish folliculin suggests requirement for embryonic brain morphogenesis.Homozygous loss of BHD causes early embryonic lethality and kidney tumor development with activation of mTORC1 and mTORC2The role of the Birt-Hogg-Dubé protein in mTOR activation and renal tumorigenesisExit from pluripotency is gated by intracellular redistribution of the bHLH transcription factor Tfe3Birt-Hogg-Dubé: tumour suppressor function and signalling dynamics central to folliculin.The SWI/SNF Protein PBRM1 Restrains VHL-Loss-Driven Clear Cell Renal Cell Carcinoma.Rapamycin ameliorates PKD resulting from conditional inactivation of Pkd1.Topical rapamycin as a treatment for fibrofolliculomas in Birt-Hogg-Dubé syndrome: a double-blind placebo-controlled randomized split-face trial.Birt-Hogg-Dubé renal tumors are genetically distinct from other renal neoplasias and are associated with up-regulation of mitochondrial gene expression.MYC activation cooperates with Vhl and Ink4a/Arf loss to induce clear cell renal cell carcinoma.Kidney-specific inactivation of Ofd1 leads to renal cystic disease associated with upregulation of the mTOR pathwayThe genetic basis of kidney cancer: a metabolic diseaseFolliculin, the product of the Birt-Hogg-Dube tumor suppressor gene, interacts with the adherens junction protein p0071 to regulate cell-cell adhesion.Folliculin regulates cell-cell adhesion, AMPK, and mTORC1 in a cell-type-specific manner in lung-derived cells.Knockdown of Slingshot 2 (SSH2) serine phosphatase induces Caspase3 activation in human carcinoma cell lines with the loss of the Birt-Hogg-Dubé tumour suppressor gene (FLCN).Human folliculin delays cell cycle progression through late S and G2/M-phases: effect of phosphorylation and tumor associated mutationsFolliculin-interacting proteins Fnip1 and Fnip2 play critical roles in kidney tumor suppression in cooperation with Flcn.Pulmonary manifestations of Birt-Hogg-Dubé syndrome.MYC oncogene overexpression drives renal cell carcinoma in a mouse model through glutamine metabolismFolate-conjugated rapamycin slows progression of polycystic kidney diseaseClinical Features, Genetics and Potential Therapeutic Approaches for Birt-Hogg-Dubé Syndrome.Characterization of a splice-site mutation in the tumor suppressor gene FLCN associated with renal cancer.The Birt-Hogg-Dubé tumor suppressor Folliculin negatively regulates ribosomal RNA synthesisFlcn-deficient renal cells are tumorigenic and sensitive to mTOR suppression.Birt-Hogg-Dubé syndrome: from gene discovery to molecularly targeted therapies.Loss of Folliculin Disrupts Hematopoietic Stem Cell Quiescence and Homeostasis Resulting in Bone Marrow Failure.Chronic AMPK activation via loss of FLCN induces functional beige adipose tissue through PGC-1α/ERRα.Tumor Suppressor Folliculin Regulates mTORC1 through Primary CiliaMutation of Fnip1 is associated with B-cell deficiency, cardiomyopathy, and elevated AMPK activity.Recruitment of folliculin to lysosomes supports the amino acid-dependent activation of Rag GTPasesAberrant epithelial morphology and persistent epidermal growth factor receptor signaling in a mouse model of renal carcinoma.
P2860
Q24298493-55DAD5BE-6404-4E6C-8BB8-6710DABEE8FAQ24298712-2E0E434A-C381-4B70-B831-CBEB8682FB25Q24321830-4DA1597B-E6F2-4D89-916D-0370BDF0AFE6Q24324012-F4C83FE9-595E-4BF3-B708-8D9B335A93F4Q24329165-7EC75423-FAD7-46CC-BB24-533CEBBEB5EAQ26785930-C37EA2AD-3F5B-47DC-B16C-E145AEF1D611Q26795505-1E91EA3B-89BE-4187-B3BE-AD81116F7CABQ26798087-F3D72889-1124-4153-878B-96CD8B661132Q26824228-6BD4AA4B-0E1B-4DE6-A274-D9F7801AAEE3Q27318467-8E6B1351-8A57-43EA-9AF3-0F9F546DA5CBQ28508427-0023A703-6C36-4904-87DE-95DB8089D475Q28590041-F1A4F161-EBFB-41DA-A028-392EED4F075EQ28594750-1FBCB90B-A73A-4138-8974-99F8630F227CQ33354615-904373E2-88FE-4B47-92C3-5CA7840AD2A4Q33683959-D6C5BD78-2F4E-4CBF-93E6-21226621EE64Q33702386-292BBB00-814B-47D4-A8E5-758BDD625957Q33728003-1E765F57-0F24-4998-AFBE-2CAABB00B2ACQ33772820-BCB5445B-2A3D-4C8B-AA3A-4BE4019A4149Q33804675-491040CD-3E0A-4D52-85EC-63B29CD72D31Q33947853-C177A420-26EE-4690-AB56-E39446EDEC6CQ34087148-F0DFFCDA-0DCF-4AD6-AB75-3688E2A15CC8Q34471857-288282F0-34CA-473D-9080-C41891EDA2C0Q34582386-E7F5442A-A1B2-47EA-8B5B-FE5BFC67B827Q34589207-E36AC55F-2A92-44B4-962D-7E27444781DFQ34846527-84619DE2-C135-427D-8083-9C5F41839800Q35279496-B797F378-2D2A-46F2-AAAD-7A7D695C195BQ35536837-0E5CE631-8909-4C38-8EDB-382BD5802335Q35669189-ACEFF52F-7352-4EC0-B09C-C13EC2D43BF5Q36280381-F3C556CF-381A-446B-8E92-362BDF2567D6Q36283086-456A7B08-7BB3-434B-B7F0-A8FF84A9D3F3Q36370579-E80E38FA-7F51-4C46-BEDA-B8CA77CCC6E3Q36477664-EDD37A44-AD43-42BF-AE54-DFE0288F8A57Q36544967-1D47039B-9A9E-4F2C-A5F3-150BB6F867B7Q36799958-EC62AFE2-8E36-4E2A-80FE-2ABF5A61C00EQ36832543-3DE23BF7-82B6-490C-AEBD-DFC478EA75FBQ36891685-795D76A7-E378-4E8D-8C88-DB2155219F9BQ36941091-0A36095A-8E03-4CAF-A5D2-BE0E922F8A22Q37065314-B49A834D-C9DA-4B74-ADA2-4B26D69CB450Q37208207-2906D8A5-7F2D-47A3-A872-60C58BFC8A8DQ37238952-5D3C301E-7C33-421A-9DC0-D33F3D9758D8
P2860
Deficiency of FLCN in mouse kidney led to development of polycystic kidneys and renal neoplasia
description
2008 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2008 թվականի հունվարին հրատարակված գիտական հոդված
@hy
article publié dans la revue scientifique PLoS ONE
@fr
artículu científicu espublizáu en 2008
@ast
scientific journal article
@en
vedecký článok (publikovaný 2008/01/01)
@sk
vědecký článek publikovaný v roce 2008
@cs
wetenschappelijk artikel (gepubliceerd op 2008/01/01)
@nl
wissenschaftlicher Artikel
@de
наукова стаття, опублікована в січні 2008
@uk
name
Deficiency of FLCN in mouse ki ...... ic kidneys and renal neoplasia
@ast
Deficiency of FLCN in mouse ki ...... ic kidneys and renal neoplasia
@en
Deficiency of FLCN in mouse ki ...... ic kidneys and renal neoplasia
@nl
type
label
Deficiency of FLCN in mouse ki ...... ic kidneys and renal neoplasia
@ast
Deficiency of FLCN in mouse ki ...... ic kidneys and renal neoplasia
@en
Deficiency of FLCN in mouse ki ...... ic kidneys and renal neoplasia
@nl
prefLabel
Deficiency of FLCN in mouse ki ...... ic kidneys and renal neoplasia
@ast
Deficiency of FLCN in mouse ki ...... ic kidneys and renal neoplasia
@en
Deficiency of FLCN in mouse ki ...... ic kidneys and renal neoplasia
@nl
P2093
P2860
P50
P3181
P1433
P1476
Deficiency of FLCN in mouse ki ...... ic kidneys and renal neoplasia
@en
P2093
Chao-Nan Qian
David Petillo
Jared Knol
Jindong Chen
Karl Dykema
Kunihiko Futami
Kyle Furge
P2860
P3181
P356
10.1371/JOURNAL.PONE.0003581
P407
P577
2008-10-30T00:00:00Z