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TFE3 Antibody

  • 货号：
    CSB-PA827304
  • 规格：
    ￥2024
  • 图片：
    • Western blot analysis of extracts from K562 cells and A549 cells, using TFE3 antibody.
    • Immunohistochemistry analysis of paraffin-embedded human brain tissue using TFE3 antibody.
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) TFE3 Polyclonal antibody
  • Uniprot No.：
    P19532
  • 基因名：
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse
  • 免疫原：
    Synthesized peptide derived from internal of Human TFE3.
  • 免疫原种属：
    Homo sapiens (Human)
  • 克隆类型：
    Polyclonal
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:3000
    IHC 1:50-1:100
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    Transcription factor that acts as a master regulator of lysosomal biogenesis and immune response. Specifically recognizes and binds E-box sequences (5'-CANNTG-3'); efficient DNA-binding requires dimerization with itself or with another MiT/TFE family member such as TFEB or MITF. Involved in the cellular response to amino acid availability by acting downstream of MTOR: in the presence of nutrients, TFE3 phosphorylation by MTOR promotes its cytosolic retention and subsequent inactivation. Upon starvation or lysosomal stress, inhibition of MTOR induces TFE3 dephosphorylation, resulting in nuclear localization and transcription factor activity. In association with TFEB, activates the expression of CD40L in T-cells, thereby playing a role in T-cell-dependent antibody responses in activated CD4(+) T-cells and thymus-dependent humoral immunity. Specifically recognizes the MUE3 box, a subset of E-boxes, present in the immunoglobulin enhancer. It also binds very well to a USF/MLTF site. May regulate lysosomal positioning in response to nutrient deprivation by promoting the expression of PIP4P1. Acts as a positive regulator of browning of adipose tissue by promoting expression of target genes; mTOR-dependent phosphorylation promotes cytoplasmic retention of TFE3 and inhibits browning of adipose tissue. Maintains the pluripotent state of embryonic stem cells by promoting the expression of genes such as ESRRB; mTOR-dependent nuclear exclusion promotes exit from pluripotency. Required to maintain the naive pluripotent state of hematopoietic stem cell; mTOR-dependent cytoplasmic retention of TFE3 promotes the exit of hematopoietic stem cell from pluripotency.
  • 基因功能参考文献：
    1. TFE3 gene rearrangement is associated with Epithelioid Rich Perivascular Epithelioid Cell Neoplasm of the Bladder. PMID: 29531006
    2. The data reveal that 4E-BP3 expression is controlled by the transcription factor TFE3 through a cis-regulatory element in the EIF4EBP3 gene promoter. PMID: 27319316
    3. A Potential G-quadruplex-forming Sequence (PQS) in the intron 2 of the TFE3 gene suggested by in silico studies was confirmed. Increased and decreased formation of G-quadruplex occurred in presence of pyridostatin and antisense oligonucleotide respectively. Stable G-quadruplex formation affected biological processes and TFE3 splicing. G-quadruplex is behind TFE3 induced oncogenesis via translocation and mRNA splicing. PMID: 29138008
    4. TFE3 may promote renal tumor growth by regulating cell cycle progression and activating the phosphatidylinositol 3kinase/AKT serine/threonine kinase 1/mTOR signaling pathway. PMID: 28713889
    5. We report for the first time the presence of both TFE3 translocation and SDHB mutation in the same renal cell carcinoma tumor. PMID: 27910947
    6. TFEB and TFE3 collaborate with each other in activated macrophages and microglia to promote efficient autophagy induction, increased lysosomal biogenesis, and transcriptional upregulation of numerous proinflammatory cytokines PMID: 27171064
    7. Findings indicated that TFE3 was an important hypoxia-induced transcriptional factor in HNSCC. PMID: 26872381
    8. results reveal that PRCC-TFE3 dual-fusion FISH probe is an efficient and concise technique for diagnosing PRCC-TFE3 RCC in paraffin-embedded tissue PMID: 28949976
    9. Case Report: Melanotic Xp11 renal cell carcinoma with ARID1B-TFE3 gene fusion. PMID: 28877054
    10. Studies identified TFEB and TFE3 as master modulators of stress response notably in the lysosomal biogenesis and autophagy with capability to upregulate hundreds of genes involved in intracellular clearance, catabolism, metabolic processes, and cellular homeostasis. PMID: 27892768
    11. Ovarian sclerosing stromal tumor strongly overexpress TFE3. PMID: 28982854
    12. Describe the unusual morphology and expanded the morphologic spectrum of SFPQ/PSF-TFE3 renal cell carcinomas. PMID: 28315422
    13. Despite TFE3 over expression, TFE3 genetic alterations are less likely to be a major molecular event driving tumorigenesis in hepatic angiolipomas. PMID: 27865795
    14. we analyze 60 Xp11 translocation cancers by fluorescence in situ hybridization using custom bacterial artificial chromosome probes to establish their TFE3 fusion gene partner PMID: 26975036
    15. These results show that ASPL-TFE3 regulates cell cycle progression and induces cellular senescence by up-regulating p21 expression. PMID: 27673450
    16. RBM10-TFE3 is a recurrent gene fusion in Xp11 translocation renal cell carcinoma. PMID: 28296677
    17. Case Report: melanotic PEComa of the sinonasal mucosa with NONO-TFE3 fusion. PMID: 28009605
    18. Xp11 translocation renal cell carcinomas with RBM10-TFE3 gene fusion demonstrating melanotic features and overlapping morphology with t(6;11) RCC. PMID: 28288037
    19. The essential parts of the Golgi stress response from the perspective of the organelle autoregulation. The pathways of the mammalian Golgi stress response have been identified, specifically the TFE3 pathway. PMID: 28179603
    20. Female patients with Xp11.2 renal cell carcinoma in adults exhibit a high incidence compared to male, but not in children. Comparable clinical characteristics including incidence of distant and lymphatic metastases, tumor stage and prognosis is presented between male and female as well as between children and adults PMID: 27401463
    21. RBM10-TFE3 fusion variant (from chromosome X paracentric inversion), therefore, appears to be a recurrent molecular event in Xp11.2 RCCs. RBM10-TFE3 fusion should be added in the list of screened fusion transcripts in targeted molecular diagnostic multiplex RT-PCR PMID: 26998913
    22. The recently identified group of Xp11.2 translocation/TFE gene fusion renal cell carcinomas (Xp11.2/TFE RCC) affect approximately 15 % of patients under 45 years old with renal cell carcinoma. PMID: 27255396
    23. Akt modulates nuclear translocation of TFE3. PMID: 28165011
    24. over-expression of TFE3 ameliorates the steatosis in hepatocytes exposed to free fatty acids (FFAs). The effects of TFE3 on hepatic steatosis are dependent on the autophagy-induced lipophagy and PGC1alpha-mediated fatty acid beta-oxidation. PMID: 26999124
    25. CT features including diameter, boundary, attenuation, nature, and circular calcification of the tumor, combined with demographic information and symptoms, may be useful to differentiate Xp11.2 RCC from different subtypes of PRCC. PMID: 26636097
    26. TFEB and TFE3 are novel components of the integrated stress response PMID: 26813791
    27. validates the utility of TFE3 break-apart fluorescence in situ hybridization on formalin-fixed paraffin-embedded tissue sections for diagnosis and confirmation of Xp11.2 translocation RCCs and alveolar soft part sarcoma PMID: 26415891
    28. Studies indicate that alveolar soft part sarcoma showed a specific chromosomal alteration, der(17)t(X:17)(p11:q25), resulting in the fusion of the TFE3 transcription factor gene (Xp11) with alveolar soft part sarcoma critical region 1 (ASPSCR1) at 17q25. PMID: 26516944
    29. TMED6-COG8 chimera might act as a novel diagnostic marker in TFE3 translocation renal cell carcinoma. PMID: 26045774
    30. We report a case of RCC with Xp11.2 translocation in a 17 year old boy who is a survivor of stage 4 neuroblastoma 13 years earlier. PMID: 25551622
    31. Case Report: renal carcinoma associated with Xp11.2 translocations/ TFE3 gene fusions diagnosed using urine cytology. PMID: 25227872
    32. Renal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE3 gene fusion (Xp11.2 RCC) was introduced as a genetically distinct entity into the World Health Organization classification of renal neoplasms in 2004. PMID: 25955396
    33. TFE3-GASE pathway is one of the regulatory pathways of the mammalian Golgi stress response PMID: 25399611
    34. PSF/SFPQ is a very common gene fusion partner in TFE3 rearrangement-associated perivascular epithelioid cell tumors (PEComas) and melanotic Xp11 translocation renal cancers. PMID: 26274027
    35. Review of the role of gene fusions involving TFE3 and TFEB in carcinogenesis in sporadic renal cell carcinoma. PMID: 25048860
    36. Case Report: PEComas of the cervix with TFE gene rearrangement. PMID: 25337301
    37. Typical Xp11-RCC manifests as an advanced, solid renal mass with mild persistent enhancement, a prevalence of intertumor hemorrhage/calcification, and a cortical epicenter location. PMID: 24136829
    38. The purpose of this study was to compare the MDCT features of renal cell carcinoma (RCC) associated with Xp11.2 translocation and TFE3 gene fusion (Xp11 RCC) and papillary RCC PMID: 25714283
    39. Report TFE3 translocation-associated perivascular epithelioid cell neoplasms of the gynecological tract. PMID: 25517951
    40. A conditional expression in mice of the fusion gene ASPSCR1-TFE3 from human alveolar soft part sarcoma (ASPS) generated a model that recapitulates the human tumor histologically and by expression profile. PMID: 25453902
    41. study presents 3 cases of perivascular epithelioid cell tumor(PEComa) with TFE3 gene abnormalities; unusual cases of PEComa with TFE3 gene rearrangement might present malignant histological features and aggressive clinical behavior PMID: 25239799
    42. Xp11.2 translocation/TFE3 gene fusion renal cell carcinoma (RCC) is the predominant form of pediatric RCC, associated with advanced stage at presentation. Patients with N + M0 maintained a favorable prognosis following surgery alone. PMID: 24726108
    43. Report TFE3 rearrangement in Xp11.2 renal cell carcinoma and alveolar soft part sarcoma. PMID: 23828314
    44. overexpression of TFE3 triggered lysosomal exocytosis and resulted in cellular clearance in a cellular model of a lysosomal storage disorder, Pompe disease, thus identifying TFE3 as a potential therapeutic target for the treatment of lysosomal disorders PMID: 24448649
    45. Expression in 16 TFE3 translocated. PMID: 24339735
    46. Case Report: bilateral renal cell carcinoma in a patient undergoing long-term dialysis, which showed false-positive immunoreactivity for TFE3 immunostaining. PMID: 24228124
    47. cutaneous PEComas consistently lack TFE3 expression PMID: 23711163
    48. In summary, we are reporting a novel subset of EHE occurring in young adults, showing a distinct phenotype and YAP1-TFE3 fusions. PMID: 23737213
    49. TFE3 FISH is highly useful in renal tumor consultation material, often resolving cases with equivocal TFE3 immunohistochemistry results. PMID: 23715164
    50. Our results support the clinical application of a TFE3 break-apart FISH assay for diagnosis and confirmation of Xp11.2 Renal cell carcinoma PMID: 23598965

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  • 相关疾病：
    A chromosomal aberration involving TFE3 is found in patients with alveolar soft part sarcoma. Translocation t(X;17)(p11;q25) with ASPSCR1 forms a ASPSCR1-TFE3 fusion protein.
  • 亚细胞定位：
    Cytoplasm, cytosol. Nucleus.
  • 蛋白家族：
    MiT/TFE family
  • 组织特异性：
    Ubiquitous in fetal and adult tissues.
  • 数据库链接：

    HGNC: 11752

    OMIM: 314310

    KEGG: hsa:7030

    STRING: 9606.ENSP00000314129

    UniGene: Hs.730740