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  4. LTBP4 Antibody, Biotin conjugated

LTBP4 Antibody, Biotin conjugated

  • 中文名稱：
    LTBP4兔多克隆抗體, Biotin偶聯(lián)
  • 貨號(hào)：
    CSB-PA02049D0Rb
  • 規(guī)格：
    ￥880
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) LTBP4 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    LTBP4
  • 別名：
    latent transforming growth factor beta binding protein 4 antibody; latent transforming growth factor beta binding protein 4L antibody; Latent-transforming growth factor beta-binding protein 4 antibody; LTBP 4 antibody; LTBP-4 antibody; LTBP4 antibody; LTBP4_HUMAN antibody; LTBP4L antibody; LTBP4S antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human Latent-transforming growth factor beta-binding protein 4 protein (977-1180AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Biotin
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA
  • Protocols：
  • 儲(chǔ)存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    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.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 功能：
    Key regulator of transforming growth factor beta (TGFB1, TGFB2 and TGFB3) that controls TGF-beta activation by maintaining it in a latent state during storage in extracellular space. Associates specifically via disulfide bonds with the Latency-associated peptide (LAP), which is the regulatory chain of TGF-beta, and regulates integrin-dependent activation of TGF-beta.
  • 基因功能參考文獻(xiàn)：
    1. DMD gene mutations involving the hinge 3 region, actin-binding domain, and exons 45-49, as well as the LTBP4 IAAM haplotype, were not associated with age of left ventricular dysfunction onset inDuchenne muscular dystrophy. PMID: 29766838
    2. High LTBP4 expression is associated with recurrence in glioblastoma. PMID: 27270107
    3. Studied the potential role of LTBP-4 in scleroderma through clinical, in vivo and in vitro studies. Results suggest that LTBP-4 protein level is increased in plasma and skin tissue of scleroderma patients; found LTBP-4 to be a potential biomarker to differentiate systemic scleroderma (SSc) from localized scleroderma (LSc) patients. PMID: 28263294
    4. The LTBP4 VTTT allele is associated with increased risk of dilated cardiomyopathy in European Americans. LTBP4 protein with the IAAM residues bound more latent TGFbeta compared to the LTBP4 VTTT protein. PMID: 26918958
    5. Our results show that LTBP4 interacts with TGFBR2 and stabilizes TGFbeta receptors by preventing their endocytosis and lysosomal degradation in a ligand-dependent and receptor kinase activity-dependent manner. PMID: 25882708
    6. We show that corticosteroid treatment and the IAAM haplotype of the LTBP4 gene are significantly associated with prolonged ambulation in patients with Duchenne muscular dystrophy PMID: 25476005
    7. In the mdx mouse model of Duchenne muscular dystrophy, the human LTBP4 transgene exacerbated muscular dystrophy symptoms and resulted in weaker muscles with an increased inflammatory infiltrate. PMID: 25338755
    8. In Caucasians with Duchenne muscular dystrophy and LTBP4 genotype there was a protective effect on age at loss of ambulation. PMID: 25641372
    9. It recruits elastin to microfibrils via fibulin-5. PMID: 25675815
    10. LTBP4 haplotype influences age at loss of ambulation, and should be considered in the management of Duchenne muscular dystrophy patients. PMID: 23440719
    11. Latent transforming growth factor beta-binding protein 4 is downregulated in esophageal cancer via promoter methylation. PMID: 23741501
    12. Data indicate mutations of FBLN4, FBLN5, and LTBP4 in 12 probands presenting with type 1 recessive cutis laxa. PMID: 22829427
    13. the G1 and G3 domains of versican were upregulated and LTBP-4 was downregulated in breast cancer stroma PMID: 21505857
    14. The lack of LTBP4-mediated targeting in malignant mammary tumor tissues may lead to a possible modification of TGF-ss1 and BMP bioavailability and function. PMID: 21468687
    15. Meta-analysis of abdominal aortic aneurysm size and growth rates demonstrated a significant association with the LTBP4 21011A>T genotype (a 2% decrease in AAA diameter, or a 0.53 mm/year reduction in AAA growth rate, per T allele [p=0.03, p=0.01]). PMID: 19897194
    16. These data suggest that LTBP-4 functions are modified by tissue-specific expression of the two N-terminally distinct variants, which in addition exhibit significant differences in cellular processing and targeting. PMID: 20175115
    17. Extracellular matrix is an important site of deposition for LTBP-3 and LTBP-4. PMID: 16157329
    18. Our findings suggest that variations in or near the HPN and LTBP4 genes do not play a role in the susceptibility to IA in the Dutch population. PMID: 18487557
    19. Novel functions for LTBP-4 as an adhesion molecule. PMID: 18585707
    20. Mutations in LTBP4 cause a syndrome of impaired pulmonary, gastrointestinal, genitourinary, musculoskeletal, and dermal development. PMID: 19836010

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  • 相關(guān)疾?。?/div>
    Urban-Rifkin-Davis syndrome (URDS); Duchenne muscular dystrophy (DMD)
  • 亞細(xì)胞定位：
    Secreted, extracellular space, extracellular matrix.
  • 蛋白家族：
    LTBP family
  • 組織特異性：
    Highly expressed in heart, skeletal muscle, pancreas, uterus, and small intestine. Weakly expressed in placenta and lung.
  • 數(shù)據(jù)庫(kù)鏈接：

    HGNC: 6717

    OMIM: 310200

    KEGG: hsa:8425

    STRING: 9606.ENSP00000311905

    UniGene: Hs.466766