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WISP3 Antibody

  • 货号：
    CSB-PA215921
  • 规格：
    ￥880
  • 图片：
    • Western blot analysis of Hela Cell Lysate using antibody. Secondary antibody was diluted at 1:20000
    • Immunohistochemical analysis of paraffin-embedded human-liver-cancer, antibody was diluted at 1:200
  • 其他：

产品详情

  • Uniprot No.：
    O95389
  • 基因名：
    WISP3
  • 别名：
    CCN 6 antibody; CCN family member 6 antibody; CCN6 antibody; CYR61 antibody; LIBC antibody; Lost in inflammatory breast cancer tumor suppressor protein antibody; MGC125987 antibody; MGC125988 antibody; MGC125989 antibody; OTTHUMP00000040421 antibody; PPAC antibody; PPD antibody; UNQ462/PRO790/PRO956 antibody; WISP 3 antibody; WISP-3 antibody; WISP3 antibody; WISP3_HUMAN antibody; WNT 1 inducible signaling pathway protein 3 antibody; Wnt 1 signaling pathway protein 3 antibody; WNT1 inducible signaling pathway protein 3 antibody; WNT1 inducible signaling pathway protein 3 precursor antibody; WNT1-inducible-signaling pathway protein 3 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Synthetic peptide from Human protein at AA range: 1-50
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 纯化方式：
    The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS, pH 7.4, containing 0.02% sodium azide as Preservative and 50% Glycerol.
  • 产品提供形式：
    Liquid
  • 应用范围：
    WB,ELISA
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-2000
    ELISA 1:10000-20000
  • 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：
    Plays a role in mitochondrial electron transport and mitochondrial respiration. Through its regulation of the mitochondrial function may play a role in normal postnatal skeletal growth and cartilage homeostasis.
  • 基因功能参考文献：
    1. WISP-3/CCN6 has a role in inhibiting apoptosis by regulating caspase pathway after hyperoxia in lung epithelial cells PMID: 29920361
    2. WISP3 gene was associated with developmental dysplasia of the hip (DDH) in Chinese Han population. GGCGG haplotype might be a biomarker for DDH. PMID: 29680248
    3. analysis of WISP3 mutations causing progressive pseudorheumatoid dysplasia in Jammu and Kashmir-India PMID: 27291587
    4. These results demonstrate that CCN6 regulates epithelial and mesenchymal states transition and tumor initiating cells programs in breast cancer PMID: 26933820
    5. two WISP3 mutations have been identified in two affected siblings by targeted NGS, thus permitting the clinical diagnosis of PPD. This case supports the utility of NGS in the genetic characterization of skeletal dysplasias, which in turn may aid their clinical care, permit predictive screening, and to provide genetic counselling to families. PMID: 26991965
    6. CCN6 acts as a molecular brake, which is appropriately balanced by Nrf2, in regulating mitochondrial function. PMID: 27252383
    7. Studies indicate that the CYR61 CTGF NOV matricellular proteins (CCN family of proteins) comprises the members CCN1, CCN2, CCN3, CCN4, CCN5 and CCN6 and have been identified in various types of cancer. PMID: 26498181
    8. WISP3 harbored not only frameshift mutation but also mutational intratumoral heterogeneity and loss of expression, which together might play a role in tumorigenesis of GC and CRC with MSI-H by inhibiting tumor suppressor functions of WISP3. PMID: 26997449
    9. WISP3 variant leads to the diagnosis of SEDT-PA. PMID: 26493744
    10. Novel and recurrent mutations in WISP3 and an atypical phenotype have been described in Indian families with progressive pseudorheumatoid dysplasia. PMID: 25988854
    11. A novel mutation c.667T>G (p.Cys223Gly) and the c.857C>G (p.Ser286*) mutation were detected in three Chinese patients with PPD. PMID: 25738435
    12. This study identified 3 different WISP3 mutations in 2 unrelated Chinese families with spondyloepiphyseal dysplasia tarda with progressive arthropathy. PMID: 25553839
    13. Suggest WISP3-Wnt/beta-catenin axis may have role in regulating gastric cancer cell proliferation and metastasis. PMID: 25400723
    14. Novel C223G and C252X mutations in exon 4 of the WISP3 gene are responsible for progressive pseudorheumatoid dysplasia in Chinese patients. PMID: 25794430
    15. Results are indicative of an operational WISP3-IGF1 regulatory loop whereby WISP3 preserves cartilage integrity by restricting IGF1-mediated hypertrophic changes in chondrocytes, at least partly, upon interaction with IGF1. PMID: 23424195
    16. A report on 11 different homozygous mutations and one instance of compound heterozygosity in the WISP3 gene in patients with progressive pseudorheumatoid dysplasia. PMID: 22987568
    17. These results reveal a novel function of the matricellular protein CCN6 and establish a mechanistic link between CCN6 and TbetaRIII in maintaining acinar organization in the breast. PMID: 23226100
    18. Mutation analysis of WISP3 allowed the confirmation of the diagnosis in 63 out of 64 typical cases of progressive pseudorheumatoid dysplasia. PMID: 22791401
    19. The data presented in this study reveal that CCN6 downregulation disrupts acinar morphogenesis and promotes invasion of mammary epithelial cells PMID: 22805309
    20. Results indicate that WNT1-inducible signaling pathway protein 3 (WISP3) mutations are associated with progressive pseudorheumatoid dysplasia (PPD). PMID: 22685593
    21. CCN6 was highly expressed in end-stage osteoarthritic cartilage, suggesting a role in cartilage homeostasis. CCN6-induced repression of ADAMTS-5 and regulation of MMP-10 expression suggest complex roles for CCN6 in cartilage biology. PMID: 22294415
    22. The mutations of the WISP3 gene in Progressive pseudorheumatoid dysplasia are located in the range of exon 2 to exon 5. PMID: 21993478
    23. demonstrated that CCN6 induced the proliferation of lung fibroblasts by binding to integrin beta1, leading to the phosphorylation of FAK(Y397). PMID: 21921419
    24. our results indicate that CCN6 enhances the migration of chondrosarcoma cells by increasing ICAM-1 expression PMID: 21391218
    25. Data define a pathway in which CCN6 attenuates IGF-1 signaling to decrease ZEB1 expression and invasion in breast cancer. PMID: 21525039
    26. KLK12 may indirectly regulate the bioavailability and activity of several growth factors through processing of their CCN binding partners PMID: 21628462
    27. Homozygous recurring mutation in WISP3 causing progressive pseudorheumatoid arthropathy. PMID: 21528827
    28. The expressions of Cyr61 and/or WISP-3 may be important biological markers in reflecting the progression, biological behaviors, metastatic potential and prognosis of NSCLC. PMID: 21159247
    29. WISPs may play important but contrasting roles in colorectal cancer with WISP-1 appearing to act as a factor stimulating aggressiveness, WISP-2 as a tumour suppressor and WISP-3 having no definable beneficial or detrimental role PMID: 20372786
    30. CCN6 (WISP3) blockade activates growth factor-independent survival and resistance to anoikis in human mammary epithelial cells PMID: 20395207
    31. WISP3 and RhoC genes expression status defines a molecular signature of inflammatory breast cancer (IBC). PMID: 20014943
    32. WISP3 acts as a tumor suppressor gene in the breast. Loss of WISP3 expression contributes to the phenotype of IBC by regulating tumor cell growth, invasion and angiogenesis. PMID: 12082632
    33. WISP3 gene expression is higher in rheumatoid arthritis synovium and fibroblast-like synoviocytes compared with osteoarthritis and normal synovial tissue and is further induced by proinflammatory cytokines in vitro. PMID: 15517620
    34. the WISP3 gene is essential to skeletal growth or homeostasis in humans but not in mice PMID: 15601861
    35. WISP transcripts may have a role in the development of human hepatocellular carcinoma PMID: 15650268
    36. stable inhibition of CCN6 expression in human mammary epithelial cells induces epithelial-mesenchymal transition, promotes anchorage-independent growth, motility and invasiveness, and sensitizes mammary epithelial cells to the growth effects of IGF-1 PMID: 16457688
    37. WISP-3 may also promote superoxide dismutase expression and activity in chondrocytes PMID: 16480948
    38. WISP3 regulates the accumulation of cellular reactive oxygen species, and mutations in WISP3 or loss of expression of WISP3 compromise this function. PMID: 17286957
    39. Mutant WISP3 triggers the phenotype shift of articular chondrocytes by promoting sensitivity to IGF1 hypothesis of osteochondrodysplasias. PMID: 17363178
    40. WISP-3 showed no statistically significant difference between groups. PMID: 17406949
    41. Results show a compound heterozygous mutation of WISP3 and a series of cellular and molecular changes disturbing the endochondral ossification in this PPD patient. PMID: 17483925
    42. These results lead to a new hypothesis that Snail and ZEB1 are downstream of CCN6 and play a critical role in CCN6-mediated regulation of E-cadherin in breast cancer. PMID: 18321996
    43. novel G46X and C114Y mutations in exon 3 in WISP3 gene are responsible for PPD in Chinese patients PMID: 19064006

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  • 相关疾病：
    Progressive pseudorheumatoid arthropathy of childhood (PPAC)
  • 亚细胞定位：
    Secreted. Mitochondrion.
  • 蛋白家族：
    CCN family
  • 组织特异性：
    Predominant expression in adult kidney and testis and fetal kidney. Weaker expression found in placenta, ovary, prostate and small intestine. Also expressed in skeletally-derived cells such as synoviocytes and articular cartilage chondrocytes.
  • 数据库链接：

    HGNC: 12771

    OMIM: 208230

    KEGG: hsa:8838

    STRING: 9606.ENSP00000354734

    UniGene: Hs.558428