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SIL1 Antibody

  • 货号：
    CSB-PA875645LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western blot
      All lanes: SIL1 antibody at 2μg/ml + Mouse kidney tissue
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 52 kDa
      Observed band size: 52 kDa
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) SIL1 Polyclonal antibody
  • Uniprot No.：
    Q9H173
  • 基因名：
  • 别名：
    BAP antibody; BiP associated protein antibody; BiP-associated protein antibody; Endoplasmic reticulum chaperone SIL 1 antibody; Endoplasmic reticulum chaperone SIL1 antibody; MSS antibody; Nucleotide exchange factor SIL 1 antibody; Nucleotide exchange factor SIL1 antibody; SIL 1 antibody; sil1 antibody; SIL1 homolog antibody; SIL1 homolog endoplasmic reticulum chaperone (S. cerevisiae) antibody; SIL1 homolog endoplasmic reticulum chaperone antibody; SIL1_HUMAN antibody; UGL 5 antibody; ULG5 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human, Mouse
  • 免疫原：
    Recombinant Human Nucleotide exchange factor SIL1 protein (32-174AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，SIL1 Antibody (CSB-PA875645LA01HU)，的标记方式是Non-conjugated。对于SIL1 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-PA875645LB01HU SIL1 Antibody, HRP conjugated ELISA
    FITC CSB-PA875645LC01HU SIL1 Antibody, FITC conjugated
    Biotin CSB-PA875645LD01HU SIL1 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, WB
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:200-1:1000
  • 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：
    Required for protein translocation and folding in the endoplasmic reticulum (ER). Functions as a nucleotide exchange factor for the ER lumenal chaperone HSPA5.
  • 基因功能参考文献：
    1. SIL1-depleted HEK293 cells are an appropriate model to identify proteins modulated by SIL1 expression level. PMID: 26468156
    2. In a child with Marinesco-Sjogren syndrome it was found that a mutation in SIL1 affected the 5' UTR, translation initiation site and the endoplasmic reticulum-targeting signal sequence. PMID: 27544240
    3. this case study is the first report on Chinese Marinesco-Sjogren syndrome (MSS) patients, MSS complicated by Dandy-Walker syndrome (DWS), and a nonstop mutation in SIL1; our findings imply the pathogenetic association between DWS and MSS PMID: 27106665
    4. Two NEFs, Grp170 and Sil1, trigger toxin release from BiP to enable successful retrotranslocation and clarify the fate of the toxin after it disengages from BiP. PMID: 25877869
    5. This study demonistrated that SIL1 mutation in patient with ataxia telangiectasia PMID: 24631270
    6. The mutations prevent SIL1 from interacting with and regulating HSPA5, leading to abnormal neuronal morphology and migration. PMID: 24473200
    7. The study confirms the previous findings of mutations in SIL1 being the major cause of Marinesco-Sjogren syndrome. PMID: 24176978
    8. The clinical features and two novel SIL1 mutations of four Dutch patients with Marinesco-Sjogren syndrome are described PMID: 23062754
    9. the very C-terminal residues of SIL1 play a role in its structural integrity rather than its localization. PMID: 22219183
    10. The patients described here manifested the cardinal features of Marinesco-Sjogren syndrome, but did not exhibit any mutation in the exons and flanking introns of the SIL1 gene. PMID: 22115007
    11. Some reported cases of Marinesco-Sjogren syndrome without base alterations in the SIL1 gene are caused by deletions rather than locus heterogeneity. PMID: 20111056
    12. Interactions between Kar2p and its nucleotide exchange factors Sil1p and Lhs1p are mechanistically distinct PMID: 20430899
    13. BAP serves as a nucleotide exchange factor for BiP PMID: 12356756
    14. Nine distinct mutations that would disrupt the SIL1 protein in individuals with Marinesco-Sjogren syndrome were identified. PMID: 16282977
    15. Four Marinesco-Sjogren syndrome-associated loss-of-function mutations in SIL1 leading to disturbed SIL1-HSPA5 interaction and protein folding were identified. PMID: 16282978
    16. A novel mutation in BAP/SIL1 gene causes Marinesco-Sjogren syndrome in an extended pedigree. PMID: 17026626
    17. SIL1 mutation is associated with Marinesco-Sjogren syndrome PMID: 18285827
    18. We sequenced the entire SIL1-coding region in 3 unrelated Japanese patients with classical MSS and identified a novel homozygous frameshift insertion mutation, 936_937insG, in exon 9 in all 3 patients. PMID: 18395226
    19. data report two novel SIL1 missense mutations in two consanguineous Pakistani families affected with Marinesco-Sjogren syndrome PMID: 19471582

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  • 相关疾病：
    Marinesco-Sjoegren syndrome (MSS)
  • 亚细胞定位：
    Endoplasmic reticulum lumen.
  • 蛋白家族：
    SIL1 family
  • 组织特异性：
    Highly expressed in tissues which produce large amounts of secreted proteins such as kidney, liver and placenta. Also expressed in colon, heart, lung, ovary, pancreas, peripheral leukocyte, prostate, spleen and thymus. Expressed at low levels throughout t
  • 数据库链接：

    HGNC: 24624

    OMIM: 248800

    KEGG: hsa:64374

    STRING: 9606.ENSP00000265195

    UniGene: Hs.483521