ArticleMolecular cancer research : MCR2019
TFE3 Xp11.2 Translocation Renal Cell Carcinoma Mouse Model Reveals Novel Therapeutic Targets and Identifies GPNMB as a Diagnostic Marker for Human Disease.
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Article in Molecular cancer research : MCR, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 54 papers.
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54 citing papers in PubMed, 85 citations in OpenAlex.
- Immunohistochemical expression of GPNMB in TFEB-rearranged and TFEB-amplified renal cell carcinomas.Virchows Archiv : an international journal of pathology · 2026Article
- GPR143, a novel immunohistochemical marker for renal tumors with FLCN/TSC/MTOR-TFE alterations.Virchows Archiv : an international journal of pathology · 2026Article
- Fusion-driven oncogenic programs shape the immune landscape in translocation renal cell carcinoma.Nature communications · 2026Article
- GPNMB-directed CAR T cell therapy against MiT/TFE-family fusion-driven solid tumors.Nature cancer · 2026Article
- Relapsed rhabdoid tumours and other non-nephroblastoma childhood and adolescent kidney tumours: perspectives from the HARMONICA collaboration.Nature reviews. Urology · 2026Review
- The Cyclin C-CDK8/19 Mediator kinase module controls PRCC-TFE3 driven senescence in renal epithelium and tumorigenesis in TFE3-RCC.Neoplasia (New York, N.Y.) · 2026Article
- Integrated Molecular, Genomic, and Clinical Characterization of Pediatric and Adolescent Translocation Renal Cell Carcinoma: A Report from the Children's Oncology Group.Biomedicines · 2026Article
- Review
- Cell-free DNA epigenomic profiling enables noninvasive detection and monitoring of translocation renal cell carcinoma.The Journal of clinical investigation · 2026Article
- SV2B Promotes the Progression of TFE3-Rearranged Renal Cell Carcinoma by Interacting with HERC2 to Impede the Degradation of NF-κB Subunits.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Article
- Comparative transcriptome atlas as an assistive modality for complex classification of rare kidney cancers.Nature communications · 2025Article
- Regulation of lipoprotein processing by GPNMB in foamy macrophages: potential therapeutic targets for atherosclerosis.Nature communications · 2025Article
- New Method Enhanced Extraction of Protein Signatures of Renal Cell Carcinoma from Proteomics Data.Journal of proteome research · 2025Article
- SFPQ-TFE3 reciprocally regulates mTORC1 and induces lineage plasticity in a mouse model of renal tumorigenesis.Nature communications · 2025Article
- LGI3 promotes the progression of TFE3-rearranged renal cell carcinoma through GEMIN6/AURKB axis.Oncogene · 2025Article
- Phenotypic screening converges on CDK9 inhibition as a therapeutic strategy in translocation renal cell carcinoma.bioRxiv : the preprint server for biology · 2025Article
- Dual Inhibition of CDK4/6 and mTORC1 Establishes a Preclinical Strategy for Translocation Renal Cell Carcinoma.bioRxiv : the preprint server for biology · 2025Article
- Article
- Glycoprotein nonmetastatic melanoma protein b immunohistochemistry can be a useful ancillary tool to diagnose subependymal giant cell astrocytoma.Virchows Archiv : an international journal of pathology · 2025Article
- TFE3 fusion oncoprotein condensates drive transcriptional reprogramming and cancer progression in translocation renal cell carcinoma.Cell reports · 2025Article
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Authors and funding
32 authors at 13 institutions in 3 countries.
Masaya BabaLaboratory of Cancer Metabolism, International Research Center for Medical Sciences (IRCMS), Kumamoto University, Kumamoto, Japan. linehanm@mail.nih.gov schmidtl@mail.nih.gov babam@kumamoto-u.ac.jp.ORCID 0000-0002-5308-6683
Mitsuko FuruyaDepartment of Molecular Pathology, Yokohama City University, Yokohama, Japan.ORCID 0000-0002-6220-8179
Takanobu MotoshimaDepartment of Urology, Kumamoto University, Kumamoto, Japan.
Martin LangUrologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.ORCID 0000-0002-0242-6713
Shintaro FunasakiLaboratory of Cancer Metabolism, International Research Center for Medical Sciences (IRCMS), Kumamoto University, Kumamoto, Japan.
Wenjuan MaLaboratory of Cancer Metabolism, International Research Center for Medical Sciences (IRCMS), Kumamoto University, Kumamoto, Japan.
Hong-Wei SunBiodata Mining and Discovery Section, NIAMS, NIH, Bethesda, Maryland.ORCID 0000-0001-9765-6505
Hisashi HasumiUrologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.ORCID 0000-0001-8211-8835
Ying HuangUrologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.
Ikuma KatoDepartment of Molecular Pathology, Yokohama City University, Yokohama, Japan.
Tsuyoshi KadomatsuDepartment of Molecular Genetics, Kumamoto University, Kumamoto, Japan.
Yorifumi SatouLaboratory of Retroviral Genomics and Transcriptomics, International Research Center for Medical Sciences (IRCMS), Center for AIDS Research, Kumamoto University, Kumamoto, Japan.ORCID 0000-0002-1495-7810
Nicole MorrisLaboratory Animal Sciences Program, Frederick National Laboratory for Cancer Research, Frederick, Maryland.
Baktiar O KarimPathology/Histotechnology Laboratory, Frederick National Laboratory for Cancer Research, Frederick, Maryland.
Lilia IlevaSmall Animal Imaging Program, Frederick National Laboratory for Cancer Research, Frederick, Maryland.ORCID 0000-0001-8286-8396
Joseph D KalenSmall Animal Imaging Program, Frederick National Laboratory for Cancer Research, Frederick, Maryland.ORCID 0000-0002-7163-4604
Luh Ade Wilan KrisnaLaboratory of Cancer Metabolism, International Research Center for Medical Sciences (IRCMS), Kumamoto University, Kumamoto, Japan.
Yukiko HasumiUrologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.ORCID 0000-0001-8211-8835
Aiko SugiyamaDSK Project, Medical Innovation Center, Kyoto University Graduate School of Medicine, Sakyo-ku, Kyoto, Japan.
Ryoma KurahashiDepartment of Urology, Kumamoto University, Kumamoto, Japan.
Koshiro NishimotoDepartment of Uro-Oncology, Saitama Medical University International Medical Center, Saitama, Japan.ORCID 0000-0002-3952-5643
Masafumi OyamaDepartment of Uro-Oncology, Saitama Medical University International Medical Center, Saitama, Japan.
Yoji NagashimaDepartment of Surgical Pathology, Tokyo Women's Medical University, Tokyo, Japan.
Naoto KurodaDepartment of Pathology, Kochi Red Cross Hospital, Kochi, Japan.
Kimi ArakiDivision of Developmental Genetics, Institute of Resource Development and Analysis, Kumamoto University, Kumamoto, Japan.
Masatoshi EtoDepartment of Urology, Kyushyu University, Fukuoka, Japan.
Masahiro YaoDepartment of Urology, Yokohama City University, Yokohama, Japan.
Tomomi KambaDepartment of Urology, Kumamoto University, Kumamoto, Japan.
Toshio SudaLaboratory of Stem Cell Regulation, International Research Center for Medical Sciences (IRCMS), Kumamoto University, Kumamoto, Japan.
Yuichi OikeDepartment of Molecular Genetics, Kumamoto University, Kumamoto, Japan.
Laura S SchmidtUrologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland. linehanm@mail.nih.gov schmidtl@mail.nih.gov babam@kumamoto-u.ac.jp.
W Marston LinehanUrologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland. linehanm@mail.nih.gov schmidtl@mail.nih.gov babam@kumamoto-u.ac.jp.
Kumamoto University · JPNational Cancer Institute · USFrederick National Laboratory for Cancer Research · USKumamoto Health Science University · JPYokohama City University · JPSaitama International Medical Center · JPJapanese Red Cross Kochi Hospital · JPKumamoto Industrial Research Institute · JPKyoto University · JPKyushu University · JPNational Institutes of Health · USNational University of Singapore · SGTokyo Women's Medical University Adachi Medical Center · JP
Funding
Molecular Therapeutics of Kidney Cancer: VHL Gene and Fumarate Hydratase GeneZIABC011038 · NCI · DIVISION OF BASIC SCIENCES - NCI · PI LINEHAN, WILLIAM MARSTON · 2009 to 2025
$29.8MHigh Throughput Next Generation Sequencing: supports genomics and epigenomics research in muscle, skin, bone and autoimmune diseases.ZICAR041207 · NIAMS · NATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASES · PI DELL'ORSO, STEFANIA · 2016 to 2025
$12.3MApplying Bioinformatics to Research in Immune, Muscle, and Bone DiseasesZIHAR041173 · NIAMS · NATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASES · PI GADINA, MASSIMO · 2009 to 2020
$11.0MTUMOR SUPPRESSOR GENES IN GENITOURINARY MALIGNANCYZ01SC006659 · SC · CLINICAL SCIENCES · PI LINEHAN, WILLIAM MARSTON · 1996 to 2007
$4.0MMolecular Therapeutics of Kidney Cancer: MET Gene and BHD GeneZ01BC011043 · NCI · DIVISION OF BASIC SCIENCES - NCI · PI LINEHAN, WILLIAM MARSTON · 2008 to 2008
$1.4MCCR NIH HHS HHSN261200800001CIntramural NIH HHS Z01 BC011043Intramural NIH HHS Z01 SC006659NCI NIH HHS HHSN261200800001E
Abstract
Renal cell carcinoma (RCC) associated with Xp11.2 translocation (TFE3-RCC) has been recently defined as a distinct subset of RCC classified by characteristic morphology and clinical presentation. The Xp11 translocations involve the TFE3 transcription factor and produce chimeric TFE3 proteins retaining the basic helix-loop-helix leucine zipper structure for dimerization and DNA binding suggesting that chimeric TFE3 proteins function as oncogenic transcription factors. Diagnostic biomarkers and effective forms of therapy for advanced cases of TFE3-RCC are as yet unavailable. To facilitate the development of molecular based diagnostic tools and targeted therapies for this aggressive kidney cancer, we generated a translocation RCC mouse model, in which the
Indexed as
Chromosomes, Human, XOncogene Proteins, FusionTranslocation, GeneticAdolescentAdultAgedAnimalsApoptosisBasic Helix-Loop-Helix Leucine Zipper Transcription FactorsBiomarkers, TumorCarcinoma, Renal CellCell Cycle ProteinsCell ProliferationChildDisease Models, AnimalFemaleBasic Helix-Loop-Helix Leucine Zipper Transcription FactorsBiomarkers, TumorCell Cycle ProteinsGPNMB protein, humanMembrane GlycoproteinsNeoplasm ProteinsOncogene Proteins, FusionPRCC protein, humanTFE3 protein, human
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