功能： 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.,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.,miscellaneous:Compared with CLCNKA/BSND, CLCNKB/BSND is more sensitive to pH and less responsive to Ca(2+).,similarity:Belongs to the chloride channel (TC 2.A.49) family.,similarity:Contains 2 CBS domains.,subunit:Interacts with BSND. Forms heteromers with BSND in the thick ascending limb of Henle and more distal segments.,tissue specificity:Expressed predominantly in the kidney.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Cell membrane; Multi-pass membrane protein.
组织表达： Expressed predominantly in the kidney.
科研货号： PLA006446
CLCKB Polyclonal Antibody
Catalog NoPLA006446
Product information
基因名称： CLCNKB
蛋白名称： Chloride channel protein ClC-Kb (Chloride channel Kb) (ClC-K2)
功能： 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.,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.,miscellaneous:Compared with CLCNKA/BSND, CLCNKB/BSND is more sensitive to pH and less responsive to Ca(2+).,similarity:Belongs to the chloride channel (TC 2.A.49) family.,similarity:Contains 2 CBS domains.,subunit:Interacts with BSND. Forms heteromers with BSND in the thick ascending limb of Henle and more distal segments.,tissue specificity:Expressed predominantly in the kidney.,
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