CLCNKB,氯离子通道KB抗体

产品编号HZ-13627R
英文名称CLCNKB
中文名称氯离子通道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.
说 明 书0.1ml 0.2ml
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,
CLCNKB,氯离子通道KB抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量75kDa
细胞定位细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
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.
CLCNKB,氯离子通道KB抗体PubMedPubMed
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.

Gene ID:
1188

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

CLCNKB,氯离子通道KB抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.