Ask about this productRelated genes to: RREB1 antibody
- Gene:
- RREB1 NIH gene
- Name:
- ras responsive element binding protein 1
- Previous symbol:
- -
- Synonyms:
- HNT
- Chromosome:
- 6p24.3
- Locus Type:
- gene with protein product
- Date approved:
- 1997-08-18
- Date modifiied:
- 2016-10-05
Related products to: RREB1 antibody
Related articles to: RREB1 antibody
- Most Spitz tumors exhibit recurrent gene fusions involving receptor tyrosine kinase or MAPK pathway genes. Here, we describe a Spitz tumor with a novel fusion involving MAP3K2. The lesion occurred on the penile shaft of an 18-year-old. Histopathologically, tumor cells were predominantly dermal, arranged in irregularly shaped nests and cords showing minimal maturation with dermal descent, and accompanied by pseudoepitheliomatous epidermal hyperplasia. Melanocytes displayed abundant eosinophilic to amphophilic cytoplasm, variably enlarged rounded nuclei, fine chromatin, and scattered nucleoli or chromocenters. There were occasional dermal mitotic figures (1/mm). The lesion involved the deep aspect of the biopsy specimen. SOX10, S100 protein, and MITF immunohistochemical stains were diffusely positive, while Melan-A and HMB45 staining was only focal. PRAME was negative. FISH showed copy number gains at 6p25 (RREB1) and 11q13 (CCND1) narrowly exceeding cutoff values. Next-generation sequencing revealed a CPEB2::MAP3K2 fusion and a low tumor mutation burden (2.1/Mb). To our knowledge, this fusion has not been previously reported in melanocytic lesions or other human tumors. A conservative re-excision was performed, and a PET/CT scan showed no evidence of dissemination. With 16 months of follow-up available, longer-term monitoring will be required to determine the clinical behavior and biologic potential of this tumor. - Source: PubMed
Publication date: 2026/04/02
McAfee John LRicotti Claudia MRonen ShiraBillings Steven Dde la Fouchardiere ArnaudKo Jennifer S - Endothelial-to-mesenchymal transition (EndMT) constitutes a transdifferentiation phenomenon during which endothelial cells (ECs) progressively acquire mesenchymal traits. Mounting evidence has established that EndMT holds a central and indispensable position in angiogenesis. Partial EndMT, an intermediate stage of ECs within the EndMT cascade, is intimately regulated by numerous enzymes. Among them, sirtuin 6 (SIRT6), a member of the sirtuin family of NAD-dependent deacetylases, has been reported to be involved in the repair of cardiovascular injury; however, the SIRT6-mediated molecular mechanism inpartial EndMT has hitherto remained largely unelucidated. In the present study, SIRT6 can regulate EndMT elicited by TGF-β and maintain a partial EndMT state. Subsequently, through conducting proteomic data analysis and performing verification using molecular biology techniques, we found that SIRT6 promoted EndMT via the upregulation of RREB1/Snail; simultaneously, SIRT6 directly targeted Sp1 to augment the expression of STC1 which in turn acetylated Smad7 to inhibit phosphorylation of Smad2/3, decreasing the formation of phosphorylated Smad2/3 and Smad4 to curtail excessive EndMT. Finally, in the model of murine hindlimb ischemia, the overexpression of Sirt6 could increase capillary density and promote the recovery of blood flow, effects that were partially abrogated by siRREB1 or recombinant STC1. These results of in vivo animal experiments are consistent with the previous conclusions of in vitro cell experiments. Collectively, our findings demonstrated that SIRT6 orchestrated a beneficial balance of the EndMT response to promote angiogenesis and mitigate ischemic injury, thereby providing a potential therapeutic target related to EndMT for ischemic diseases. - Source: PubMed
Publication date: 2026/03/20
Zhang ZhentaoFang ZheyanZhao ShuangAbdurahman MukaddasZhao GangFeng RunyangGuo ZhenyangYu XuetingHong HangnanGeng JilongNie XiansuAbuduwahapi SupuyaLan LingyunGe JunboLi Hua - Glioma stem-like cells (GSCs), characterized by self-renewal capacity, therapeutic resistance, and high tumorigenicity, are considered the fundamental drivers of glioma aggressiveness and recurrence. However, the core regulatory mechanisms underlying GSCs stemness maintenance remain unclear. In this study, we identified that the NIBAN2 is highly expressed in glioma tissues and GSCs, and its expression is closely associated with poor patient prognosis. Functional experiments demonstrated that NIBAN2 directly binds to Flightless I (FLII) and enhances its interaction with the transcription factor Ras-responsive element-binding protein 1 (RREB1), thereby promoting nuclear translocation. Together, the NIBAN2-FLII-RREB1 complex activates the Toll-like receptor (TLR3) signaling pathway, sustaining the stem-like phenotype and tumorigenic potential of GSCs. Further investigation revealed that RREB1 transcriptionally upregulates NIBAN2 and CD44, promoting the expression of the key glycolytic enzyme LDHA (Lactate Dehydrogenase A). This establishes a feed-forward signaling-transcription-metabolism axis driven by NIBAN2/FLII/RREB1, facilitating metabolic reprogramming. Multi-omics and metabolomic analyses confirmed that this loop enhances glycolytic flux to maintain GSCs' metabolic homeostasis. Drug screening identified the HIV protease inhibitor nelfinavir as a specific disruptor of the NIBAN2-FLII complex. When combined with the LDHA inhibitor FX11, it induced synergistic anti-tumor effects in organoid and patient-derived xenograft models, thereby significantly inhibiting tumor progression and prolonging survival. Clinical samples further confirmed the co-upregulation of NIBAN2, FLII, and RREB1 in GBM (Glioblastoma) tissues, which correlates with SOX2 and Ki-67 expression and poor prognosis. Collectively, this study, for the first time, reveals that NIBAN2 maintains GSCs stemness by activating FLII-RREB1 axis and TLR3 signaling, thereby establishing a feed-forward signaling-transcription-metabolism axis. This finding provides a novel strategy and potential targets for precise therapeutic intervention against glioma stemness. - Source: PubMed
Publication date: 2026/02/25
Shi Liang LiangQiao XinweiHu YueWang MinjieYu ShaojieGong ZihanRao YuxinZheng HuiChen SitingLi LinFu RongLiang TaoYao JinJiang XiaobingLi Junjun - Activated regulatory T cells (aTregs) exhibit potent immunosuppressive functions and can migrate to tissues, playing a crucial role in attenuating inflammatory responses. The precise role and molecular mechanisms through which Notch2 regulates the immunoregulatory functions of aTregs remain incompletely elucidated. Here, we elucidate the mechanisms through which Notch2 influences aTreg dynamics and mitigates allergic rhinitis (AR) development. Mechanistically, the targeted knockout of Notch2 in aTregs resulted in decreased Foxo1 expression and elevated phosphorylated ASC (p-ASC) levels, culminating in GSDMD-N-mediated pyroptosis in aTregs. Moreover, the Notch2 intracellular domain (NICD2) promoted the nuclear translocation of RREB1 through direct protein-protein interactions, thereby enhancing Foxo1 transcriptional activity. Importantly, the adoptive transfer of Notch2+ aTregs significantly reduced Th2 inflammatory responses in AR mice, providing an effective therapeutic strategy for managing AR-related inflammation. Overall, our findings establish a novel paradigm in the pathogenesis of AR in which Notch2 expression dictates the functional dichotomy of aTregs in maintaining their immunoregulatory capacity or undergoing inflammatory pyroptosis. - Source: PubMed
Publication date: 2026/01/12
Qiao Yue-LongXu ShanZou YouYang RuiZheng HanLiu Jia-YuFan Hui-MingZhang Yuan-YuanKong Yong-GangJiao Wo-ErChen Shi-Ming - RREB1::MRTFB fusion-positive extra-glossal mesenchymal neoplasm is a recently recognized tumor so far mostly described in the head and neck area and in the mediastinum. At least some of these neoplasms are potentially related to ectomesenchymal chondromyxoid tumor of the tongue since they share an identical gene fusion and overlapping morphological features in some cases. Herein we describe for the first time two cases with RREB1::MRTFB fusion located in the skin and subcutis. The cases occurred in 36-year-old male with a cutaneous mass on the nose and in 65-year-old woman with a large subcutaneous mass involving the lower leg. Histopathologically, both cases consisted of bland ovoid cells in a myxoid stroma. Immunohistochemically, one of the two cases showed diffuse S100 positivity. RREB1::MRTFB fusion was confirmed in both cases. In summary, the two reported cases expand the anatomical spectrum and improve our understanding of this rare emerging entity. - Source: PubMed
Publication date: 2025/12/26
Důra MiroslavBaněčková MartinaRozkoš TomášMichal MichalMichal MichaelKastnerová Liubov