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FREM1 Antibody

  • 货号：
    CSB-PA686013LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • IHC image of CSB-PA686013LA01HU diluted at 1:500 and staining in paraffin-embedded human tonsil tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) FREM1 Polyclonal antibody
  • Uniprot No.：
    Q5H8C1
  • 基因名：
    FREM1
  • 别名：
    BC037594 antibody; BNAR antibody; C9orf143 antibody; C9orf145 antibody; C9orf154 antibody; D430009N09 antibody; D630008K06 antibody; FLJ25461 antibody; FRAS1-related extracellular matrix protein 1 antibody; FREM 1 antibody; FREM1 antibody; FREM1_HUMAN antibody; Heb antibody; MOTA antibody; Protein QBRICK antibody; QBRICK antibody; RGD1306981 antibody; RP11-265B7.2 antibody; RP23-410K19.1 antibody; TILRR antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human FRAS1-related extracellular matrix protein 1 protein (1957-2082AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，FREM1 Antibody (CSB-PA686013LA01HU)，的标记方式是Non-conjugated。对于FREM1 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA686013LB01HU FREM1 Antibody, HRP conjugated ELISA
    FITC CSB-PA686013LC01HU FREM1 Antibody, FITC conjugated
    Biotin CSB-PA686013LD01HU FREM1 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:500-1:1000
  • 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：
    Extracellular matrix protein that plays a role in epidermal differentiation and is required for epidermal adhesion during embryonic development.
  • 基因功能参考文献：
    1. we identified genome-wide significant association involving measures of central upper lip height at 9p22 within FREM1 PMID: 28441456
    2. FREM1 expression is significantly downregulated in human masticatory mucosa during wound healing PMID: 28005267
    3. this report describes a patient with a phenotype compatible with Manitoba-oculo-tricho-anal syndrome in whom two novel FREM1 mutations were identified in the compound heterozygous state thus broadening the mutational spectrum of the disease. PMID: 28111185
    4. Disruption of the FREM1 gene can produce a spectrum of clinical manifestations encompassing the previously described MOTA and BNAR syndromes. PMID: 23401257
    5. We conclude that FREM1 plays a critical role in the development of the diaphragm and that FREM1 deficiency can cause CDH in both humans and mice. PMID: 23221805
    6. The significant association of rs1552896 with an HIV-resistant phenotype, together with the expression profile of FREM1 in tissues relevant to HIV infection, suggests that FREM1 is a potentially novel candidate gene for resistance to HIV infection. PMID: 22915813
    7. The location of the IBD region 16 kb from FREM1 suggests the phenotype in Manitoba oculotrichoanal syndrome patients is attributable to a variant outside of FREM1, potentially in a regulatory element. PMID: 22690109
    8. FREM1 encodes a basement membrane protein of FRAS1-related extracellular matrix protein 1 which is required for epidermal adhesion during embryonic development PMID: 22876578
    9. a role for TILRR in selective amplification of NF-kappaB responses through IL-1RI and suggest that the specificity is determined by changes in receptor conformation and adapter protein recruitment. PMID: 22262840
    10. These data suggest that copy number variations and mutations involving FREM1 can be identified in a significant percentage of people with MC with or without midface hypoplasia. PMID: 21931569
    11. Manitoba-oculo-tricho-anal (MOTA) syndrome is caused by mutations in FREM1. FREM1 deficiency ties the molecular cause of MOTA syndrome closely to the pathogenesis of Fraser syndrome. PMID: 21507892
    12. TILRR, an isoform encoded by an alternatively spliced FREM1 mRNA, is an IL-1RI co-receptor that associates with the signaling receptor complex to enhance recruitment of MyD88 and control Ras-dependent amplification of NF-kappaB and inflammatory responses. PMID: 19940113
    13. The phenotypic variability reported for different Frem1 mouse mutants suggests that the apparently distinct phenotype of bifid nose and anorectal and renal anomalies syndrome in humans may represent a previously unrecognized variant of Fraser syndrome. PMID: 19732862

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  • 相关疾病：
    Bifid nose, with or without anorectal and renal anomalies (BNAR); Manitoba oculotrichoanal syndrome (MOTA); Trigonocephaly 2 (TRIGNO2)
  • 亚细胞定位：
    Secreted, extracellular space, extracellular matrix, basement membrane.
  • 蛋白家族：
    FRAS1 family
  • 数据库链接：

    HGNC: 23399

    OMIM: 248450

    KEGG: hsa:158326

    STRING: 9606.ENSP00000370262

    UniGene: Hs.50850