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  4. EFEMP2 Antibody

EFEMP2 Antibody

  • 货号：
    CSB-PA009079
  • 规格：
    ￥880
  • 其他：

产品详情

  • Uniprot No.：
    O95967
  • 基因名：
    EFEMP2
  • 别名：
    EFEMP 2 antibody; EFEMP2 antibody; EGF containing fibulin like extracellular matrix protein 2 antibody; EGF-containing fibulin-like extracellular matrix protein 2 antibody; FBLN 4 antibody; FBLN4 antibody; FBLN4_HUMAN antibody; FIBL 4 antibody; FIBL-4 antibody; FIBL4 antibody; Fibulin 4 antibody; Fibulin-4 antibody; MBP 1 antibody; MBP1 antibody; Mutant p53 binding protein 1 antibody; Protein UPH1 antibody; UPH 1 antibody; UPH1 antibody; UPH1 protein antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Synthesized peptide derived from the Internal region of Human Fibulin-4.
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 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.
  • 保存缓冲液：
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 产品提供形式：
    Liquid
  • 应用范围：
    WB, IHC, IF, ELISA
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:2000
    IHC 1:100-1:300
    IF 1:200-1:1000
    ELISA 1:10000
  • 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 crucial role in elastic fiber formation in tissue, and in the formation of ultrastructural connections between elastic laminae and smooth muscle cells in the aorta, therefore participates in terminal differentiation and maturation of smooth muscle cell (SMC) and in the mechanical properties and wall integrity maintenance of the aorta. In addition, is involved in the control of collagen fibril assembly in tissue throught proteolytic activation of LOX leading to cross- linking of collagen and elastin. Also promotes ELN coacervation and participates in the deposition of ELN coacervates on to microfibrils but also regulates ELN cross- linking through LOX interaction. Moreover adheres to the cells through heparin binding in a calcium-dependent manner and regulates vascularlar smooth muscle cells proliferation through angiotensin signaling.
  • 基因功能参考文献：
    1. different mutations in the fibulin-4 gene result in different molecular defects affecting secretion rates, protein stability, LOX-induced cross-linking, or binding to other ECM components and molecules of the TGF-beta pathway. PMID: 27339457
    2. the expression of fibulin-4 was negatively correlated with the malignant phenotype of endometrial cancer cells. Fibulin-4 may have the ability to suppress endometrial cancer cell invasion and proliferation PMID: 28177909
    3. The results revealed that fibulin-4 expression was upregulated in osteosarcoma, and was positively correlated with low differentiation, lymph node metastasis, and poor prognosis. Fibulin-4 was also found to be over-expressed in highly invasive cell lines and in the highly invasive subclones PMID: 28339091
    4. Findings suggested that fibulin-4 is important for the proteolytic activation of lysyl oxidase which has a pivotal role in cross-linking of collagen and elastin. PMID: 26690653
    5. Data indicates that Fibulin-4 is a novel gene that is found overexpressed in ovarian cancer and associated with poor prognostic clinicopathologic features. PMID: 25885889
    6. pulmonary emphysema in aneurysmal Fibulin-4 deficient (Fibulin-4(R)) mice PMID: 25255451
    7. Homozygous mutations in exon 7 of the FBLN4 gene can produce lethal vasculopathy. PMID: 24838734
    8. This study demonstrated that fibulin-4 may serve as a new prognostic factor and as a potential therapeutic target for patients with cervical carcinoma. PMID: 24737201
    9. The expression level of EFEMP2 is dramatically increased in colorectal cancer patients, even at the early stage, compared with healthy controls. PMID: 22506683
    10. Studied Fibulin-4 expression in aortic wall to find out its role in aortic dissection development. Used samples of aortic wall from 10 patients operated for acute ascending aortic dissection and five patients for chronic ascending aortic dissection. PMID: 23518852
    11. A lethal, genetic disorder characterized by severe deformation of elastic arteries, was linked to novel mutations in the FBLN4 gene. PMID: 22943132
    12. Low EFEMP2 expression is associated with malignant pleural mesothelioma. PMID: 23313295
    13. EFEMP2 is a novel PITX2-interacting protein that may bear importance for the development of anterior segment dysgenesis (ASD) and glaucoma. PMID: 22919265
    14. FBLN4 has 10 coding exons PMID: 22070778
    15. In this largest cohort of reported patients with a mutated EFEMP2 gene, we illustrate the phenotypic spectrum of inherited AA due to a novel EFEMP2 mutation. PMID: 22440127
    16. patients with recessive FBLN4 mutations are predominantly characterized by aortic aneurysms, arterial tortuosity and stenosis; evidence for the involvement of altered TGFbeta signaling in the pathogenesis of FBLN4 mutations PMID: 20389311
    17. Fibulin-4, expressed in chondrocytes and recognized as an autoantigen mainly in osteoarthritis (OA) rather than in rheumatoid arthritis, may play pathogenic roles in OA. PMID: 16493080
    18. Fibulin-4, considered as a structural protein, may also participate in regulating elastic-fibre formation in human cells through the regulation of tropoelastin expression. PMID: 19627254

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  • 相关疾病：
    Cutis laxa, autosomal recessive, 1B (ARCL1B)
  • 亚细胞定位：
    Secreted, extracellular space, extracellular matrix. Secreted, extracellular space, extracellular matrix, basement membrane.
  • 蛋白家族：
    Fibulin family
  • 数据库链接：

    HGNC: 3219

    OMIM: 604633

    KEGG: hsa:30008

    STRING: 9606.ENSP00000309953

    UniGene: Hs.731454