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LAMB2 Antibody

  • 货号：
    CSB-PA958054
  • 规格：
    ￥2024
  • 图片：
    • Western blot analysis of extracts from RAW264.7 cells, using LAMB2 antibody.
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) LAMB2 Polyclonal antibody
  • Uniprot No.：
    P55268
  • 基因名：
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Synthesized peptide derived from internal of Human LAMB2.
  • 免疫原种属：
    Homo sapiens (Human)
  • 克隆类型：
    Polyclonal
  • 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.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:3000
  • 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：
    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.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Pierson syndrome (PIERSS); Nephrotic syndrome 5 with or without ocular abnormalities (NPHS5)
  • 亚细胞定位：
    Secreted, extracellular space, extracellular matrix, basement membrane. Note=S-laminin is concentrated in the synaptic cleft of the neuromuscular junction.
  • 数据库链接：

    HGNC: 6487

    OMIM: 150325

    KEGG: hsa:3913

    STRING: 9606.ENSP00000307156

    UniGene: Hs.439726