Rabbit Anti-CLCNKB/FITC Conjugated antibody

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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

Cat:

SL13627R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Dog,Pig,Cow,Rabbit,)

Immunogen：

KLH conjugated synthetic peptide derived from human CLCNKB

Format：

Lyophilized or Liquid

Storage instructions：

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 ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

75kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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. CLSLCKA is a kidney-specific chloride channel that mediates transepithelial chloride transport in the thin ascending limb of the Henle loop in the inner medulla. CLSLCKA plays a crucial role in urine concentration. The gene encoding human CLSLCKA 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. CLSLCKB mediates basolateral chloride ion efflux in the thick ascending limb and in more distal nephron segments. The gene encoding human CLSLCKB 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: P5361 Human

SwissProt: Q9WUB6 Mouse

SwissProt: P5362 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.

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