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SCN1B Antibody

  • 货号：
    CSB-PA020835LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • IHC image of CSB-PA020835LA01HU diluted at 1:300 and staining in paraffin-embedded human heart 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.
    • Immunofluorescence staining of A549 cells with CSB-PA020835LA01HU at 1:100, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) SCN1B Polyclonal antibody
  • Uniprot No.：
    Q07699
  • 基因名：
    SCN1B
  • 别名：
    GEFSP1 antibody; SCN1B antibody; SCN1B_HUMAN antibody; sodium channel beta 1 subunit antibody; Sodium channel subunit beta 1 antibody; Sodium channel subunit beta-1 antibody; Sodium channel voltage gated type I beta antibody; Sodium channel voltage gated type I beta subunit antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Sodium channel subunit beta-1 protein (44-156AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，SCN1B Antibody (CSB-PA020835LA01HU)，的标记方式是Non-conjugated。对于SCN1B 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-PA020835LB01HU SCN1B Antibody, HRP conjugated ELISA
    FITC CSB-PA020835LC01HU SCN1B Antibody, FITC conjugated
    Biotin CSB-PA020835LD01HU SCN1B 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, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:200-1:500
    IF 1:50-1:200
  • 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：
    Regulatory subunit of multiple voltage-gated sodium channel complexes that play important roles in excitable membranes in brain, heart and skeletal muscle. Enhances the presence of the pore-forming alpha subunit at the cell surface and modulates channel gating characteristics and the rate of channel inactivation. Modulates the activity of multiple pore-forming alpha subunits, such as SCN1A, SCN2A, SCN3A, SCN4A, SCN5A and SCN10A.; Cell adhesion molecule that plays a critical role in neuronal migration and pathfinding during brain development. Stimulates neurite outgrowth. Has no regulatory function on the SCN2A sodium channel complex.
  • 基因功能参考文献：
    1. we confirm the recessive inheritance of 2 novel SCN1B mutations in 5 children from 3 families with developmental epileptic encephalopathy. The recessive inheritance and early death in these patients is consistent with the Dravet-like phenotype observed in Scn1b(-/-) mice. PMID: 28218389
    2. This report provides the first genetic evidence of SCN1B mutation causing the Benign Familial Infantile Epilepsy (BFIE) phenotype. PMID: 28566192
    3. Contribution of Cardiac Sodium Channel beta-Subunit Variants to Brugada Syndrome. PMID: 26179811
    4. SCN1B gene mutations that reduce sodium channel current may provide a mechanistic link between Atrioventricular nodal reentrant tachycardia and Brugada syndrome and predispose to expression of both phenotypes. PMID: 25998140
    5. In a nonreferred nationwide Danish cohort of SIDS cases, up to 5/66 (7.5%) of SIDS cases can be explained by genetic variants in the sodium channel complex genes. PMID: 25757662
    6. We also identified an SCN1B T189M variant in 2 probands with lone AF and in 1 of 250 control subjects. PMID: 26129877
    7. Study showed that the human SCN1B C121W epilepsy mutation leads to decreased axon initial segments expression of SCN1B in heterozygous CW mice and a complete lack of SCN1B in homozygous WW mice PMID: 25421039
    8. data revealed SCN1Bb as a susceptibility gene responsible for LQTS PMID: 24662403
    9. High SCN1B expression is associated with increased tumour growth and metastasis in breast cancer. PMID: 24729314
    10. Hippocampal networks of a NaV beta1 transgenic mouse model of genetic epilepsy show enhanced excitability. PMID: 24605816
    11. experimental data indicate that sodium channel voltage-gated type I beta subunit (Navbeta1b)/H162P results in reduced sodium channel activity functionally affecting the ventricular action potential. PMID: 24561865
    12. SCN1B mutation is not a common cause of Dravet syndrome. PMID: 23182416
    13. results suggest that R214Q variation in SCN1Bb is a functional polymorphism that may serve as a modifier of the substrate responsible for Brugada syndrome or SIDS phenotypes PMID: 22155597
    14. Our study supports the association of SCN1Bb with BrS. PMID: 22284586
    15. A novel seizure-causing mechanism is suggested for NaV1.2beta1 in patients harboring mutant C121W subunit: increased channel excitability at elevated temperature. PMID: 22292491
    16. SCN1B is the gene responsible in one out of six Tunisian families with febrile seizures (FS) that may contribute susceptibility for the five others. PMID: 21040232
    17. This study demonstrated that SCN1B may not be related to the occurrence of benign partial epilepsy in infancy or convulsions with gastroenteritis. PMID: 21882141
    18. The results suggested that beta1B p.G257R may contribute to epilepsy through a mechanism that includes intracellular retention resulting in aberrant neuronal pathfinding. PMID: 21994374
    19. Enhanced tubulin polymerization reduces sarcolemmal Na(v)1.5 expression and I(Na) amplitude in a beta1-subunit-independent fashion and causes I(Na) fast and slow inactivation impairment in a beta1-subunit-dependent way PMID: 19861310
    20. The IVS3+ 2996(TTA)8 allele in SCN1B commonly seen in Japanese would not be pathogenic itself but may render male, middle-aged Japanese more susceptible to Brugada syndrome PMID: 20137763
    21. Mutation of the sodium channel subunit SCN1B linked again to generalized epilepsy with febrile seizures PMID: 12011299
    22. Functional and biochemical analysis of a sodium channel beta1 subunit mutation responsible for generalized epilepsy with febrile seizures plus type 1. PMID: 12486163
    23. the expression of NaCh beta1 subunit protein in astrocytes is plastic, and indicate a novel mechanism for modulation of glial function in gliosis-associated pathologies. PMID: 12677453
    24. The IVS2-2A>C transition deletes AA 70-74 in the central hydrophobic core of the extracellular Ig domain, disrupting the hydrophobic interaction in the Ig-like fold & proper [beta]1 folding & causing a persistent inward Na+ current & hyperexcitability. PMID: 14504340
    25. identification and characterization of a novel splicing variant; functional studies in oocytes demonstrate that the beta1B subunit increases the ionic current when coexpressed with the tetrodotoxin sensitive channel, NaV1.2 PMID: 14622265
    26. Although data suggest that SCN1B activity does not directly influence membrane potential, intracellular Ca(2+) release, or proliferation in normal human pulmonary artery smooth muscle cells, its physiological functions remain unresolved. PMID: 16052353
    27. Use of these Na+ channel models in simple neuron models revealed that both mutations(R85C, R85H) cause an increase in excitability but the R85H mutation was more excitable. PMID: 17604911
    28. In summary, the mutant beta1 subunits essentially fail to modulate alpha subunits which could increase neuronal excitability and underlie GEFS+ pathogenesis. PMID: 17629415
    29. This suggests that mutations in the SCN1B gene is not a prevalent cause of familial cases of FS and epilepsy or GEFS+ in Scandinavia. PMID: 17927801
    30. SCN1B mutations were not found to directly cause long QT syndrome. PMID: 18052691
    31. In patient with epilepsy were carried the mutation (C121W) of SCN1B. PMID: 18093548
    32. Febrile Seizure is not related to the most common mutations of SCN1B in two Tunisian families PMID: 18175077
    33. SCN1B may have a role in human arrhythmia susceptibility PMID: 18464934
    34. The data of this study suggested that SCN1B p.R125C is an autosomal recessive cause of Dravet syndrome through functional gene inactivation. PMID: 19710327
    35. Loss of function mutations in sodium channel beta-subunits were identified in patients with atrial fibrillation and were associated with a distinctive ECG phenotype PMID: 19808477

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  • 相关疾病：
    Generalized epilepsy with febrile seizures plus 1 (GEFS+1); Brugada syndrome 5 (BRGDA5); Atrial fibrillation, familial, 13 (ATFB13); Epileptic encephalopathy, early infantile, 52 (EIEE52)
  • 亚细胞定位：
    [Isoform 1]: Cell membrane; Single-pass type I membrane protein. Perikaryon. Cell projection. Cell projection, axon.; [Isoform 2]: Perikaryon. Cell projection. Secreted.
  • 蛋白家族：
    Sodium channel auxiliary subunit SCN1B (TC 8.A.17) family
  • 组织特异性：
    The overall expression of isoform 1 and isoform 2 is very similar. Isoform 1 is abundantly expressed in skeletal muscle, heart and brain. Isoform 2 is highly expressed in brain and skeletal muscle and present at a very low level in heart, placenta, lung,
  • 数据库链接：

    HGNC: 10586

    OMIM: 600235

    KEGG: hsa:6324

    STRING: 9606.ENSP00000396915

    UniGene: Hs.436646