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  4. SUFU Antibody

SUFU Antibody

  • 货号：
    CSB-PA954231
  • 规格：
    ￥1100
  • 图片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using CSB-PA954231(SUFU Antibody) at dilution 1/10, on the right is treated with fusion protein. (Original magnification: ×200)
    • The image on the left is immunohistochemistry of paraffin-embedded Human colon cancer tissue using CSB-PA954231(SUFU Antibody) at dilution 1/10, on the right is treated with fusion protein. (Original magnification: ×200)
    • Gel: 10%SDS-PAGE, Lysate: 50 μg, Lane: Jurkat cells, Primary antibody: CSB-PA954231(SUFU Antibody) at dilution 1/100, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 10 seconds
  • 其他：

产品详情

  • Uniprot No.：
    Q9UMX1
  • 基因名：
  • 别名：
    OTTHUMP00000020374 antibody; OTTHUMP00000020377 antibody; OTTHUMP00000020379 antibody; PRO1280 antibody; SU FU antibody; SU(F)U antibody; Su(fu) antibody; SUFU antibody; SUFU negative regulator of hedgehog signaling antibody; SUFU_HUMAN antibody; SUFUH antibody; SUFUXL antibody; Suppressor of Fused antibody; Suppressor of fused homolog (Drosophila) antibody; Suppressor of fused homolog antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse
  • 免疫原：
    Fusion protein of Human SUFU
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen affinity purification
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:1000-1:2000
    WB 1:200-1:1000
    IHC 1:15-1:50
  • 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：
    Negative regulator in the hedgehog/smoothened signaling pathway. Down-regulates GLI1-mediated transactivation of target genes. Down-regulates GLI2-mediated transactivation of target genes. Part of a corepressor complex that acts on DNA-bound GLI1. May also act by linking GLI1 to BTRC and thereby targeting GLI1 to degradation by the proteasome. Sequesters GLI1, GLI2 and GLI3 in the cytoplasm, this effect is overcome by binding of STK36 to both SUFU and a GLI protein. Negative regulator of beta-catenin signaling. Regulates the formation of either the repressor form (GLI3R) or the activator form (GLI3A) of the full-length form of GLI3 (GLI3FL). GLI3FL is complexed with SUFU in the cytoplasm and is maintained in a neutral state. Without the Hh signal, the SUFU-GLI3 complex is recruited to cilia, leading to the efficient processing of GLI3FL into GLI3R. When Hh signaling is initiated, SUFU dissociates from GLI3FL and the latter translocates to the nucleus, where it is phosphorylated, destabilized, and converted to a transcriptional activator (GLI3A). Required for normal embryonic development. Required for the proper formation of hair follicles and the control of epidermal differentiation.
  • 基因功能参考文献：
    1. Down-regulation of microRNA-224 inhibits growth and epithelial-to-mesenchymal transition phenotype via modulating SUFU expression in bladder cancer cells. PMID: 28780419
    2. Itch/beta-arrestin2 complex binds SuFu and induces its Lys63-linked polyubiquitylation without affecting its stability. PMID: 29515120
    3. Patients with SUFU pathogenic variants were significantly more likely to develop a medulloblastoma, a meningioma or an ovarian fibroma, but were less likely to develop a jaw cyst. PMID: 28596197
    4. Nek2A is found to stabilize SuFu at least partly depending on its kinase activity, thereby triggering phosphorylation of the SuFu protein. PMID: 27297360
    5. Extra-mitochondrial prosurvival BCL-2 proteins regulate gene transcription by inhibiting the SUFU tumour suppressor. PMID: 28945232
    6. Missense mutation in SUFU gene is associated with Joubert Syndrome with Cranio-facial and Skeletal Defects. PMID: 28965847
    7. In conclusion, this study showed the potential of miR-342-3p as a therapeutic target to promote bone regeneration by modulating expression of Sufu in UCMSCs. PMID: 28765042
    8. miRNA-194 is oncogenic and promotes gastric cancer cell proliferation and migration by activating Wnt signaling, at least in part, via suppression of SUFU. PMID: 27810403
    9. SUFU's role in Hedgehog signaling, tumor progression, and highlight a way in which BCCs can arise PMID: 28030567
    10. In summary, these findings reveal Fbxl17 as a novel regulator of the Hedgehog signaling pathway and highlight the perturbation of the Fbxl17-Sufu axis in the pathogenesis of medulloblastoma. PMID: 27234298
    11. there was a positive correlation between VDR status and the expression of Suppressor of fused gene (SuFu), a hedgehog pathway inhibitor. miR-214 on the other hand suppressed SuFu protein expression. PMID: 27693451
    12. The data indicate that there exists a novel transcript variant of SUFU which can be transcribed and translated into corresponding protein and its transcription is related with metastasis of lymph nodes in pancreatic ductal adenocarcinoma. PMID: 27840902
    13. We showed that the supplementation of the osteogenic differentiation medium with PTHrP inhibited the alkaline phosphatase activity and the expression of the transcription factor DLX3, but the depletion of PTHrP did not support the differentiation of DFCs.We showed that SUFU (Suppressor Of Fused Homolog) was not regulated during the osteogenic differentiation in DFCs PMID: 27368119
    14. This study uncovers a previously unappreciated miR-214-Sufu pathway in controlling EMT and metastasis of lung adenocarcinoma. PMID: 26462018
    15. A germline SUFU mutation was present in a patient with MHIBCC, and additional acquired SUFU mutations underlie the development of infundibulocystic basal cell carcinomas PMID: 26677003
    16. SUFU germline polymorphism is associated with acute GVHD PMID: 26067905
    17. Sufu has a role in repressing Gli1 transcription and nuclear accumulation, inhibiting glioma cell proliferation, invasion and vasculogenic mimicry, improving glioma chemo-sensitivity and prognosis PMID: 25373737
    18. suggest childhood brain magnetic resonance imaging surveillance is justified in SUFU-related, but not PTCH1-related, Gorlin syndrome PMID: 25403219
    19. SUFU polymorphisms are associated with neural tube defects in a high-risk population in China. PMID: 24070372
    20. Functionally, RIOK3 acts as a SUFU-dependent positive regulator of Hedgehog signaling. PMID: 24018050
    21. Data indicate a significant role of hedgehog receptor PTCH1 and SUFU in the pathogenesis of keratocystic odontogenic tumor (KCOT). PMID: 23951062
    22. It is demonstrated that GLI binding is associated with major conformational changes in SUFU, including an intrinsically disordered loop that is also crucial for pathway activation. PMID: 24311597
    23. The 'closed' form of Sufu is stabilized by Gli binding and inhibited by Hh treatment, whereas the 'open' state of Sufu is promoted by Gli-dissociation and Hh signaling. PMID: 24217340
    24. Polymorphisms in the SUFU gene (encoding for a negative regulator of the hedgehog signaling pathway) are associated with protection from Enterobacteriacea bacteremia related organ injury and sepsis severity. PMID: 23538333
    25. SUFU germline mutation is associated with nevoid basal cell carcinoma syndrome associated with meningioma. PMID: 22829011
    26. Our genetic and functional analyses indicate that germline mutations in SUFU also predispose to meningiomas, particularly to multiple meningiomas. PMID: 22958902
    27. mechanism of action on Hedgehog signaling by a suppressor of fused carboxy terminal variant PMID: 22666390
    28. germline SUFU mutations are responsible for a high proportion of desmoplastic medulloblastoma in children younger than 3 years of age PMID: 22508808
    29. Rab23 directly associates with Su(Fu) and inhibits Gli1 function in a Su(Fu)-dependent manner. PMID: 22365972
    30. inactivating germline mutations of SUFU cause ~2-3% of sporadic medulloblastomas and > 10% of desmoplastic medulloblastomas. PMID: 21188540
    31. We found that hedgehog-interacting protein, PDGFRalpha, smoothened and Su(Fu) gene highly expressed in the primary esophageal squamous cell carcinomas PMID: 21210262
    32. Glis3 interacts with Suppressor of Fused (SUFU) PMID: 21543335
    33. ciliary localization of Sufu is dependent on ciliary-localized Gli proteins, and is inhibited by PKA activation PMID: 21209912
    34. Dual Phosphorylation of suppressor of fused (Sufu) by PKA and GSK3beta regulates its stability and localization in the primary cilium. PMID: 21317289
    35. Suppressor of fused transgene negatively regulates hedgehog signaling and proliferation in retinal progenitor cells (RPCs); unexpectedly, Sufu-null RPCs fail to maintain their multipotency, differentiating as amacrine and horizontal neurons. PMID: 21451052
    36. Germline SUFU mutations were identified in two families with several children under 3 years of age diagnosed with medulloblastoma. PMID: 19833601
    37. Suppressor of Fused represses Gli-mediated transcription by recruiting the SAP18-mSin3 corepressor complex. PMID: 11960000
    38. Mutations in SUFU predispose to medulloblastoma. PMID: 12068298
    39. loss of SUFU function results in overactivity of both the Sonic Hedgehog, and the WNT signaling pathways, leading to excessive proliferation and failure to differentiate resulting in medulloblastomas. PMID: 15077159
    40. Overexpression of SUFU is associated with Split-hand/split-foot malformation 3 PMID: 16691619
    41. A role for SIL in derepressing GLI1 from the negative control of SUFU. PMID: 18829525
    42. Shh signaling regulates Sufu activity by inducing its turnover via the ubiquitin-proteasome system. PMID: 18997815
    43. expression induced by 4-Hydroxynonenal,one of several lipid oxidation products, in human leukemia cells, HL-60; this tumor suppressor protein is a target of miR-378 PMID: 19022373
    44. human tumor suppressor SUFU has a role in Hedgehog signaling [review] PMID: 19055941
    45. Suppressor of fused was overexpressed in endometrial carcinoma compared with the hyperplastic endometrium PMID: 19432668
    46. First report of a germ line SUFU mutation associated with Gorlin syndrome. PMID: 19533801

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  • 相关疾病：
    Medulloblastoma (MDB); Joubert syndrome 32 (JBTS32)
  • 亚细胞定位：
    Cytoplasm. Nucleus.
  • 蛋白家族：
    SUFU family
  • 组织特异性：
    Ubiquitous in adult tissues. Detected in osteoblasts of the perichondrium in the developing limb of 12-week old embryos. Isoform 1 is detected in fetal brain, lung, kidney and testis. Isoform 2 is detected in fetal testis, and at much lower levels in feta
  • 数据库链接：

    HGNC: 16466

    OMIM: 155255

    KEGG: hsa:51684

    STRING: 9606.ENSP00000358918

    UniGene: Hs.404089