| 供应商 | 上海联迈生物工程有限公司 |
| 数量 | 大量 |
| 靶点 | 详见说明书 |
| 级别 | 1 |
| 目录编号 | LM-13627R-FITC |
| 克隆性 | 多克隆 |
| 抗原来源 | Rabbit |
| 保质期 | 1年 |
| 抗体英文名 | Anti-CLCNKB/FITC |
| 抗体名 | Anti-CLCNKB/FITC |
| 标记物 | FITC标记 |
| 宿主 | Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, |
| 适应物种 | Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, |
| 免疫原 | 详见说明书 |
| 亚型 | IGg |
| 形态 | 粉末、液体、冻干粉 |
| 应用范围 | ICC=1:50-200 IF=1:50-200 |
| 浓度 | 1mg/ml |
| 保存条件 | -20 °C |
| 规格 | 100ul |
| 英文名称 | Anti-CLCNKB/FITC |
| 中文名称 | FITC标记的氯离子通道KB抗体 |
| 别 名 | Bartter syndrome type 3; Chloride channel Kb; Chloride channel kidney B; Chloride channel protein ClC-Kb; Chloride channel voltage sensitive Kb; ClC K2; ClC-K2; ClCK2; CLCKB; CLCKB_HUMAN; CLCNKB; hClC Kb; hClCKb; MGC24087; OTTHUMP00000011120; OTTHUMP00000011121; RP11 5P18.8. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 神经生物学 信号转导 通道蛋白 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 75kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human CLCNKB |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | background: The family of voltage-dependent chloride channels (CLCs) regulate cellular trafficking of chloride ions, a critical component of all living cells. CLCs regulate excitability in muscle and nerve cells, aid in organic solute transport, and maintain cellular volume. CLC-KA is a kidney-specific chloride channel that mediates transepithelial chloride transport in the thin ascending limb of the Henle loop in the inner medulla. CLC-KA plays a crucial role in urine concentration. The gene encoding human CLC-KA maps to chromosome 1p36. Mutations in this gene may be associated with nephrogenic diabetes insipidus in those cases where mutations in the vasopressin V2 receptor and the AQP2 water channel are lacking. CLC-KB mediates basolateral chloride ion efflux in the thick ascending limb and in more distal nephron segments. The gene encoding human CLC-KB maps to chromosome 1p36. Mutations in this gene cause type III Barter’s syndrome which is characterized by renal salt-wasting and low blood pressure. Function: 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. Subcellular Location: Cell membrane. Tissue Specificity: Expressed predominantly in the kidney. DISEASE: Defects in CLCNKB are the cause of Bartter syndrome type 3 (BS3) [MIM:607364]; also known as classic Bartter syndrome. It is an autosomal recessive form of often severe intravascular volume depletion due to renal salt-wasting associated with low blood pressure, hypokalemic alkalosis, hypercalciuria, and normal serum magnesium levels. Defects in CLCNKB are a cause of Bartter syndrome type 4B (BS4B) [MIM:613090]. A digenic, recessive disorder characterized by impaired salt reabsorption in the thick ascending loop of Henle with pronounced salt wasting, hypokalemic metabolic alkalosis, and varying degrees of hypercalciuria. Bartter syndrome type 4B is associated with sensorineural deafness. Similarity: Belongs to the chloride channel (TC 2.A.49) family. CLCNKB subfamily. Contains 2 CBS domains. Database links: Entrez Gene: 1188 Human Entrez Gene: 56365 Mouse Entrez Gene: 79430 Rat Omim: 602023 Human SwissProt: P51801 Human SwissProt: Q9WUB6 Mouse SwissProt: P51802 Rat Unigene: 352243 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
