Salt-losing nephropathy in mice with a null mutation of the Clcnk2 gene
| Autor: | Hayo Castrop, Bernhard Gess, Ina Maria Schießl, Katharina Fremter, Alexandra Grill, Anna Hammer |
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| Rok vydání: | 2016 |
| Předmět: |
0301 basic medicine
medicine.medical_specialty Physiology 030204 cardiovascular system & hematology Kidney Bartter syndrome Dinoprostone Renin-Angiotensin System Mice 03 medical and health sciences 0302 clinical medicine Chloride Channels Internal medicine medicine Animals Distal convoluted tubule Mice Knockout CLCNKB biology urogenital system Reabsorption Bartter Syndrome medicine.disease Bartter's syndrome Phenotype 030104 developmental biology medicine.anatomical_structure Endocrinology Renal sodium excretion Mutation biology.protein Urine osmolality Glomerular Filtration Rate |
| Zdroj: | Acta Physiologica. 218:198-211 |
| ISSN: | 1748-1708 |
| DOI: | 10.1111/apha.12755 |
| Popis: | Aim The basolateral chloride channel ClC-Kb facilitates Cl reabsorption in the distal nephron of the human kidney. Functional mutations in CLCNKB are associated with Bartter's syndrome type 3, a hereditary salt-losing nephropathy. To address the function of ClC-K2 in vivo, we generated ClC-K2-deficient mice. Methods ClC-K2-deficient mice were generated using TALEN technology. Results ClC-K2-deficient mice were viable and born in a Mendelian ratio. ClC-K2−/− mice showed no gross anatomical abnormalities, but they were growth retarded. The 24-h urine volume was increased in ClC-K2−/− mice (4.4 ± 0.6 compared with 0.9 ± 0.2 mL per 24 h in wild-type littermates; P = 0.001). Accordingly, ambient urine osmolarity was markedly reduced (590 ± 39 vs. 2216 ± 132 mosmol L−1 in wild types; P |
| Databáze: | OpenAIRE |
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