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Gene expression data from FLCN-restored UOK257-2 renal cancer cells

ABSTRACT: UOK257 cell line was derived from a BHD patient. It harbors a germline mutation in FLCN (c.1285dupC) and LOH. UOK257-2 cells were generated from UOK257 cells by introducing wildtype FLCN using retrovirus. FLCN inactivation induces TFE3 transcriptional activity by increasing its nuclear localization. Thus expression microarray was used to identify the genes regulated by FLCN and TFE3. UOK257-2 cells expressing wildtype FLCN were transfected with 1) scrambled, 2) FLCN, 3) TFE3 and 4)FLCN/TFE3 siRNAs with Lipofectamine 2000 transfection reagent. Cells were harvested 3 days after transfection and RNAs were isolated using Trizol reagent and RNeasy Mini Kit. The genes induced by FLCN siRNA and reduced by TFE3 siRNA were chosen as a candidate genes regulated by FLCN and TFE3.

ORGANISM(S): Homo sapiens

SUBMITTER: William Linehan

PROVIDER: E-GEOD-23614 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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The UOK 257 cell line: a novel model for studies of the human Birt-Hogg-Dubé gene pathway.

Yang Youfeng Y Padilla-Nash Hesed M HM Vira Manish A MA Abu-Asab Mones S MS Val Daniel D Worrell Robert R Tsokos Maria M Merino Maria J MJ Pavlovich Christian P CP Ried Thomas T Linehan W Marston WM Vocke Cathy D CD

Cancer genetics and cytogenetics 20080101 2

The establishment, characterization, and tumorigenicity of a new epithelial cell line (UOK 257) derived from human renal carcinoma of an individual with Birt-Hogg-Dubé (BHD) syndrome are reported. Unlike other established renal tumor cell lines from sporadic renal cell carcinoma, this is the first established renal tumor cell line of BHD, an inheritable neoplastic syndrome. The isolated tumor cells display loss of contact inhibition in vitro, and produce subcutaneous tumors in mouse xenografts. ...[more]

PMID: 18206534

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Gene expression data from FLCN-restored UOK257-2 renal cancer cells

Project description:UOK257 cell line was derived from a BHD patient. It harbors a germline mutation in FLCN (c.1285dupC) and LOH. UOK257-2 cells were generated from UOK257 cells by introducing wildtype FLCN using retrovirus. FLCN inactivation induces TFE3 transcriptional activity by increasing its nuclear localization. Thus expression microarray was used to identify the genes regulated by FLCN and TFE3.

2010-10-11 | GSE23614 | GEO

TFEB AND TFE3 DRIVE KIDNEY CYSTOGENESIS AND TUMORIGENESIS

Project description:Birt-Hogg-Dubè (BHD) syndrome is an inherited condition caused by loss-of-function mutations in the gene encoding the tumor-suppressor protein folliculin (FLCN) and frequently associated with kidney cysts and cancer. FLCN acts as a negative regulator of TFEB and TFE3 transcription factors, master controllers of lysosomal biogenesis and autophagy, by enabling their phosphorylation by the mechanistic Target Of Rapamycin Complex 1 (mTORC1). We previously showed that deletion of TFEB rescued the renal cystic phenotype of kidney-specific Flcn KO mice. Using Flcn/TFEB/TFE3 double and triple KO mice we now show that both TFEB and TFE3 contribute, in a differential and cooperative manner, to kidney cystogenesis. Importantly, silencing of either TFEB or TFE3 rescued tumorigenesis in patient-derived xenografts (PDXs) generated from a kidney tumor of a BHD patient. Furthermore, transcriptome analyses performed in transgenic mice, PDXs and patient tumor samples revealed TFEB/TFE3 downstream targets that may contribute to their tumorigenic activity. Our findings demonstrate in disease-relevant models that TFEB and TFE3 are key drivers of kidney tumorigenesis and suggest novel therapeutic strategies based on the inhibition of these transcription factors.

2023-04-04 | PXD035450 | Pride

Loss of FLCN-FNIP1/2 Induces a Non-Canonical Interferon 1 Response in Human Renal Tubular Epithelial Cells

Project description:Germline inactivating mutations in Folliculin (FLCN) cause Birt–Hogg–Dubé (BHD) syndrome, a rare autosomal dominant disorder predisposing to kidney tumors. FLCN is a conserved, essential gene that has been linked to diverse cellular processes but the mechanisms by which FLCN prevents kidney cancer remain unknown Here we show that FLCN loss activates E-box target genes in human renal tubular epithelial cells (RPTEC/TERT1), including RRAGD, yet without modifying mTORC1 activity. Surprisingly, inactivation of FLCN or its binding partners FNIP1/FNIP2 activates interferon response genes but independently of interferon. Mechanistically, FLCN loss promotes recruitment of STAT2 to chromatin and slows cellular proliferation. Our integrated analysis identifies STAT1/2 as a novel target of FLCN in renal cells and BHD tumors. STAT1/2 activation appears to counterbalance TFE3-directed hyper-proliferation and may influence the immune response. These findings shed light on unique roles of FLCN in human renal tumorigenesis and pinpoint novel prognostic biomarkers.

2021-02-11 | PXD021346 | Pride

Short interfering RNAs (siRNAs)-based knockdown of OLFM2 or PKP2 in human adipocytes

Project description:We introduced into subcutaneous fully-differentiated human adipocytes from the same subject short interfering RNAs (siRNAs), thus enabling the study of functional consequences of OLFM2 (siOLFM2; n=3 biological replicates) and PKP2 (siPKP2; n=3) knockdown in these cells. A lipid-based transfection reagent (i.e. Lipofectamine 3000 reagent) was used to provide a transient (72h), convenient, reliable and efficient transfection into in vitro cultured and fully differentiated adipocytes. A non-targeting siRNA was used as control (siCTRL; n=4) for both modifications.

2021-12-28 | GSE182231 | GEO

Tfe3-bound regions in naive pluripotent mouse embryonic stem (ES) cells

Project description:The bHLH transcription factor Tfe3 is a powerful regulator of pluripotency and we report a genome-wide analysis of Tfe3 occupancy in mouse ES cells. Nuclear localization of Tfe3 is inhibited by a protein complex containing the tumor-suppressor Folliculin (Flcn) and we also determine Tfe3 binding sites in ES cells expressing an shRNA targeting Flcn. Specificity is controlled for by using unspecific IgGs and ES cells expressing an shRNA targeting Tfe3. ChIP-Seq profiling of Tfe3 in ES cells

2013-04-10 | E-GEOD-39815 | biostudies-arrayexpress

Gene expression profiling of BHDS-associated renal tumors

Project description:Renal tumors that arise in individuals with BHD Syndrome represent a molecularly distinct form of renal cancer. In addition, BHD syndrome is due to a mutation the folliculin gene (FLCN). While the folliculin gene is an important tumor suppressor gene, the molecular function of this gene is not well defined. By analyzing tumor samples that contain FLCN mutations, we demonstrate that the FLCN gene is an important regulator of mitochondrial function. Gene expression profiling of BHDS tumors and normal kidney tissues

2010-12-21 | E-GEOD-21816 | biostudies-arrayexpress

Gene expression profiling of BHDS-associated renal tumors

2010-12-21 | GSE21816 | GEO

Characterizing the tumor suppressor activity of FLCN in Birt-Hogg-Dube syndrome through transcriptiomic and proteomic analysis

Project description:Birt-Hogg-Dube (BHD) syndrome patients are uniquely susceptible to all renal tumour subtypes. The underlying mechanism of carcinogenesis is unclear. To study cancer development in BHD, we used human proximal kidney (HK2) cells with short-term and long-term folliculin (FLCN) knockdown. HK2 cells lacking FLCN had an altered transcriptome profile with cell cycle control gene enrichment. G1/S cell cycle checkpoint signaling was compromised with heightened protein levels of cyclin D1 (CCND1) and hyperphosphorylation of retinoblastoma 1 (RB1). Taken together with our proteomic work, our findings indicate that long-term FLCN loss and associated cell cycle defects in BHD patients could contribute to their increased risk of cancer.

2024-11-28 | GSE283021 | GEO

Mus musculus

Project description:Flcn and BHD renal tumorigenesis

| PRJNA213999 | ENA

Gene expression microarray analysis following siRNA knockdown of T-UC.300A

Project description:SH-SY5Y cells were transfected with an siRNA against T-UC.300A (final concentration 50nM) or negative control siRNA (Dharmacon Negative Control #1, final concentration 50 nM) using the transfection reagent Lipofectamine (Invitrogen). Media was changed after 24hrs. RNA was extracted 120hrs after transfection. Gene expression microarray analysis was carried out using Roche NimbleGens 4x72K Homo Sapiens gene expression array.

2012-10-14 | E-MEXP-3712 | biostudies-arrayexpress

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