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CLCNKB Antibody

  • 货号：
    CSB-PA005488LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: Hela whole cell lysate, Rat kidney tissue
      All lanes: CLCNKB antibody at 3µg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 76, 57 kDa
      Observed band size: 76 kDa
    • IHC image of CSB-PA005488LA01HU diluted at 1:300 and staining in paraffin-embedded human breast 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.
    • Immunofluorescence staining of HepG2 cells with CSB-PA005488LA01HU 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) CLCNKB Polyclonal antibody
  • Uniprot No.：
    P51801
  • 基因名：
    CLCNKB
  • 别名：
    Bartter syndrome type 3 antibody; Chloride channel Kb antibody; Chloride channel kidney B antibody; Chloride channel protein ClC-Kb antibody; Chloride channel voltage sensitive Kb antibody; ClC K2 antibody; ClC-K2 antibody; ClCK2 antibody; CLCKB antibody; CLCKB_HUMAN antibody; CLCNKB antibody; hClC Kb antibody; hClCKb antibody; MGC24087 antibody; OTTHUMP00000011120 antibody; OTTHUMP00000011121 antibody; RP11 5P18.8 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human, Rat
  • 免疫原：
    Recombinant Human Chloride channel protein ClC-Kb protein (538-687AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，CLCNKB Antibody (CSB-PA005488LA01HU)，的标记方式是Non-conjugated。对于CLCNKB 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-PA005488LB01HU CLCNKB Antibody, HRP conjugated ELISA
    FITC CSB-PA005488LC01HU CLCNKB Antibody, FITC conjugated
    Biotin CSB-PA005488LD01HU CLCNKB 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, IF
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    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：
    Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport. May be important in urinary concentrating mechanisms.
  • 基因功能参考文献：
    1. Taking advantage of the largest number of functional results of CLCNKB mutations, we reveal the functionally important domains and severe mutational spots of the hClC-Kb channel and establish the genotype-phenotype association in classic Bartter's Syndrome. PMID: 28555925
    2. Five patients had 1 or more mutations in CLCNKB, of whom 3 had homozygous mutations and 2 had single heterozygous mutations and only in CLCNKB had hypocalciuria. PMID: 26770037
    3. Single loci of tag Single Nucleotide Polymorphisms of CLCNKA_B are not enough to increase the Essential Hypertension susceptibility, the combination of CLCNKA SNP, salt, marine products, meat, edible oil consumption is associated with elevated risk PMID: 25919862
    4. results demonstrate that the carboxyl terminus of hClC-Kb is not part of the binding site for barttin, but functionally modifies the interplay with barttin. PMID: 26453302
    5. These results demonstrate that mutations in a cluster of hydrophobic residues within transmembrane domain 1 affect barttin-CLC-K interaction and impair gating modification by the accessory subunit PMID: 26063802
    6. we report here for the first time that ClC-Kb disease-causing mutations located around the selectivity filter can result in both reduced surface expression and hyperactivity in heterologous expression systems PMID: 24271511
    7. Case Report: 2 mutations in the CLCNKB gene, leading to a molecular diagnosis of Bartter syndrome type III in case of sudden infant death. PMID: 25923035
    8. study investigated the functional consequences of seven mutations; four mutants carried no current whereas others displayed a 30-60 percent reduction in conductance as compared with wild-type ClC-Kb PMID: 23703872
    9. expands the association between CLCNKB and essential hypertension to a non-European ancestry population PMID: 22578033
    10. This article presents the case of a patient with hypokalaemia caused by CLCNKB gene mutation hard to categorise as GS or BS type 3. PMID: 23345488
    11. CLCNKB mutations are associated with Bartter syndrome. PMID: 21865213
    12. novel missense variant of the CLCNKB gene in two patients with type III Bartter syndrome PMID: 21479528
    13. there was no significant association between the SLC12A3 R904Q variant and the ClC-Kb-T481S variant and essential hypertension in Mongolian and Han populations in Inner Mongolia PMID: 21644212
    14. four mutations in the CLCNKB gene, among patients suffering from bartter and Gitelman syndromes PMID: 21631963
    15. Three novel CLCNKB mutations are identified associated with classic Bartter syndrome with a role in altering the functional properties of ClC-Kb channels. PMID: 19807735
    16. DNA mutational analysis of CLCNKB in Bartter syndrome type 3. PMID: 11865110
    17. presence of Gitelman and Bartter syndrome and CBS phenotypes, in a kindred with the CLCNKB R438H mutation. PMID: 12472765
    18. CLCKB expression is demonstrated in stria vascularis, spiral ligament and limbal fibrocytes, interdental cells and satellite cells of spiral ganglion neurons of mice harboring enhanced green fluorescence protein gene driven by the human CLCKB promoter. PMID: 14502078
    19. Genetic heterogeneity of ClC-Kb chloride channels correlates with functional heterogeneity, which assigns ClC-Kb to a set of genes potentially relevant for polygenic salt-sensitivity of blood pressure regulation. PMID: 14675050
    20. The mutation ClC-Kb(T481S) of the renal epithelial Cl- channel ClC-Kb strongly activates ClC-Kb chloride channel function in vitro and may predispose to the development of essential hypertension in vivo. PMID: 15148291
    21. There is no association with hypertension of CLCNKB polymorphism at a hypertension locus on chromosome 1p36. PMID: 16003175
    22. confirms a weak genotype-phenotype correlation in patients with CLCNKB mutations and supports the founder effect of the A204T mutation in Spain PMID: 16391491
    23. Identification of a novel mutation of the CLCNKB gene, DeltaL130 associated with Bartter syndrome. PMID: 16902263
    24. Results identify large heterozygous deletion mutations in the CLCNKB gene in patients with type III Bartter syndrome. PMID: 17622951
    25. roles of Thr418Ser polymorphism of the CLCNKB gene and Arg904Gln polymorphism in the TSC gene on essential hypertension need to be explored in other ethnic groups PMID: 17997379
    26. In a large cohort of ante/neonatal Bartter syndrome, deafness, transient hyperkalaemia and severe hypokalaemic hypochloraemic alkalosis orientate molecular investigations to BSND, KCNJ1 and CLCNKB genes, respectively. PMID: 19096086
    27. Threonine change to serine at position 481 in CLCNKB is associated with essential hypertension in males within the Ghanaian population. PMID: 19226700

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  • 相关疾病：
    Bartter syndrome 3 (BARTS3); Bartter syndrome 4B, neonatal, with sensorineural deafness (BARTS4B)
  • 亚细胞定位：
    Cell membrane; Multi-pass membrane protein.
  • 蛋白家族：
    Chloride channel (TC 2.A.49) family, CLCNKB subfamily
  • 组织特异性：
    Expressed predominantly in the kidney.
  • 数据库链接：

    HGNC: 2027

    OMIM: 602023

    KEGG: hsa:1188

    STRING: 9606.ENSP00000364831

    UniGene: Hs.352243