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BRAT1 Antibody

  • 货号：
    CSB-PA757659LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: Jurkat whole cell lysate, PC3 whole cell lysate, MCF-7 whole cell lysate, K562 whole cell lysate, A549 whole cell lysate
      All lanes: BRAT1 antibody at 1:2000
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 89, 31, 32 kDa
      Observed band size: 89 kDa
    • IHC image of CSB-PA757659LA01HU diluted at 1:300 and staining in paraffin-embedded human kidney 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.
    • IHC image of CSB-PA757659LA01HU diluted at 1:300 and staining in paraffin-embedded human colon cancer 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) BRAT1 Polyclonal antibody
  • Uniprot No.：
    Q6PJG6
  • 基因名：
    BRAT1
  • 别名：
    BAAT 1 antibody; BAAT1 antibody; BRAT 1 antibody; brat1 antibody; BRAT1_HUMAN antibody; BRCA1-associated ATM activator 1 antibody; BRCA1-associated protein required for ATM activation protein 1 antibody; BRCA1-associated protein required for ATM activation-1 antibody; C7orf27 antibody; chromosome 7 open reading frame 27 antibody; HEAT repeat containing protein C7orf27 antibody; MGC22916 antibody; RMFSL antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human BRCA1-associated ATM activator 1 protein (603-821AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，BRAT1 Antibody (CSB-PA757659LA01HU)，的标记方式是Non-conjugated。对于BRAT1 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-PA757659LB01HU BRAT1 Antibody, HRP conjugated ELISA
    FITC CSB-PA757659LC01HU BRAT1 Antibody, FITC conjugated
    Biotin CSB-PA757659LD01HU BRAT1 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, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:1000-1:5000
    IHC 1:200-1:500
  • 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：
    Involved in DNA damage response; activates kinases ATM, SMC1A and PRKDC by modulating their phosphorylation status following ionizing radiation (IR) stress. Plays a role in regulating mitochondrial function and cell proliferation. Required for protein stability of MTOR and MTOR-related proteins, and cell cycle progress by growth factors.
  • 基因功能参考文献：
    1. Biallelic sequence variants in BRAT1 have been reported to cause a variety of ocular and systemic manifestations, but to our knowledge, this is the first report of inner retinal dysfunction manifest as selective loss of full-field ERG scotopic and photopic b-wave amplitudes. PMID: 28635423
    2. compared with RMFSL, BRAT1 mutations can result in both moderately severe presentations evident by later-onset epilepsy and survival past infancy, as well as milder presentations that include intellectual disability, ataxia/dyspraxia, and cerebellar atrophy. PMID: 27282546
    3. our data expand the clinical symptoms and demonstrate variability in the natural clinical course of BRAT1-associated phenotypes. Patients with early onset seizures, postnatal microcephaly, feeding problems, and muscular hypertonia or contractures should hence be screened for BRAT1 mutations. PMID: 27282648
    4. we report two affected siblings with compound heterozygous truncating mutations in BRAT1 and intra-familial phenotypic heterogeneity, with a less severe disease course in the female sibling. This phenotypic variability should be taken into account when treating patients with BRAT1-associated neurodegenerative disease. PMID: 27480663
    5. We conclude that BRAT1 should be added to the growing list of genes related to early-onset severe encephalopathy with epilepsy. PMID: 26535877
    6. Our results further support that mutations of BRAT1 could lead to epileptic encephalopathy. PMID: 25319849
    7. Ndfip1 is required during stress for ubiquitinating and trafficking BRAT1 into the nucleus. PMID: 25631046
    8. Findings suggest novel roles of BRAT1 in cell proliferation and mitochondrial functions. PMID: 25070371
    9. Data on crystal structure of BRCA1 binding with phosphopeptides suggest that C-terminal domain of BRCA1 interacts with BAAT1 and ATRIP with preferences for specific side chains; in BAAT1, phospho-Ser269 and Phe272 are the main interacting residues. PMID: 24073851
    10. Study identified sequence variants in the known disease-causing genes SLC6A3 and FLVCR1, and present evidence to strongly support the pathogenicity of variants identified in TUBGCP6, BRAT1, SNIP1, CRADD, and HARS. PMID: 22279524

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  • 相关疾病：
    Rigidity and multifocal seizure syndrome, lethal neonatal (RMFSL)
  • 亚细胞定位：
    Nucleus. Cytoplasm.
  • 组织特异性：
    Ubiquitously expressed.
  • 数据库链接：

    HGNC: 21701

    OMIM: 614498

    KEGG: hsa:221927

    STRING: 9606.ENSP00000339637

    UniGene: Hs.520623