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LAMB2 Antibody

  • 中文名稱：
    LAMB2兔多克隆抗體
  • 貨號：
    CSB-PA14279A0Rb
  • 規(guī)格：
    ￥440
  • 促銷：
    小規(guī)格抗體限時一口價
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) LAMB2 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
  • 別名：
    Lamb2 antibody; LAMB2_HUMAN antibody; Laminin B1s chain antibody; Laminin beta 2 antibody; laminin S antibody; Laminin subunit beta 2 antibody; Laminin subunit beta-2 antibody; laminin; beta 2 (laminin S) antibody; Laminin-11 subunit beta antibody; Laminin-14 subunit beta antibody; Laminin-15 subunit beta antibody; Laminin-3 subunit beta antibody; Laminin-4 subunit beta antibody; Laminin-7 subunit beta antibody; Laminin-9 subunit beta antibody; LAMS antibody; NPHS5 antibody; S laminin antibody; S-LAM beta antibody; S-laminin subunit beta antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human Laminin subunit beta-2 protein (1530-1798AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated

    本頁面中的產(chǎn)品，LAMB2 Antibody (CSB-PA14279A0Rb)，的標記方式是Non-conjugated。對于LAMB2 Antibody，我們還提供其他標記。見下表：

    可提供標記
    標記方式 貨號 產(chǎn)品名稱 應(yīng)用
    HRP CSB-PA14279B0Rb LAMB2 Antibody, HRP conjugated ELISA
    FITC CSB-PA14279C0Rb LAMB2 Antibody, FITC conjugated
    Biotin CSB-PA14279D0Rb LAMB2 Antibody, Biotin conjugated ELISA
  • 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)品評價

靶點詳情

  • 功能：
    Binding to cells via a high affinity receptor, laminin is thought to mediate the attachment, migration and organization of cells into tissues during embryonic development by interacting with other extracellular matrix components.
  • 基因功能參考文獻：
    1. In conclusion, we reported three Chinese cases with different LAMB2 mutations and different phenotypes, further broadening the range of eye and kidney pathology associated with mutations in LAMB2. PMID: 27925579
    2. No pathogenic LAMB2 mutations were found in the cohort of children with steroid-resistant focal segmental glomerulosclerosis. PMID: 24856380
    3. Comprehensive gene sequencing revealed a novel LAMB2 variant (c.440A --> G; His147R) that was homozygous in the 9 living, affected family members, observed at a frequency of 2.1% in the Old Order Mennonite population, and absent in 91 non-Mennonite controls PMID: 21236492
    4. Novel mutations in steroid-resistant nephrotic syndrome diagnosed in Tunisian children were detected in LAMB2. PMID: 21125408
    5. All previously reported and several novel LAMB2 mutations in relation to the associated phenotype in patients from 39 unrelated families, are reviewed. PMID: 20556798
    6. Deficiency in LAMB2 causes congenital nephrosis with mesangial sclerosis and distinct eye abnormalities. PMID: 15367484
    7. Hepalaminin, an autoantigen from chronic hepatitis C, consists of two domains of laminin beta-2 and a specific domain. PMID: 15603881
    8. Mutations in the LAMB2 gene encoding laminin beta2, a component of the glomerular basement membrane and the neuro-muscular junction are responsible for the characteristic renal and eye abnormalities of Pierson syndrome. PMID: 16898484
    9. Recessive missense mutations in LAMB2 expand the clinical spectrum of LAMB2-associated disorders. PMID: 16912710
    10. LAMB2 has to be considered as culprit of milder disorders including nephrosis and variable ocular anomalies. PMID: 16921188
    11. We demonstrated that overabundance of the beta2 chain of laminin is associated with increased basement membrane thickness and is possibly related to spermatogenic dysfunction PMID: 17804866
    12. Milder phenotypes of Pearson Syndrome may be related to hypomorphic LAMB2 alleles. PMID: 17943323
    13. Pierson syndrome is defined by the association of mental retardation, microcoria and DMS caused by mutation in LAMB2 gene PMID: 18065803
    14. Study excluded LAMB2 as a candidate gene for Galloway-Mowat syndrome. PMID: 18594871
    15. Loss-of-function mutations in laminin beta2 (LAMB2) cause a broad range of ocular pathology, emphasizing the importance of laminin beta2 in eye development. PMID: 18672223

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  • 相關(guān)疾?。?/div>
    Pierson syndrome (PIERSS); Nephrotic syndrome 5 with or without ocular abnormalities (NPHS5)
  • 亞細胞定位：
    Secreted, extracellular space, extracellular matrix, basement membrane. Note=S-laminin is concentrated in the synaptic cleft of the neuromuscular junction.
  • 數(shù)據(jù)庫鏈接：

    HGNC: 6487

    OMIM: 150325

    KEGG: hsa:3913

    STRING: 9606.ENSP00000307156

    UniGene: Hs.439726