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WTC deafness Kyoto (dfk): a rat model for extensive investigations of Kcnq1 functions.

Authors: Gohma, H  Kuramoto, T  Kuwamura, M  Okajima, R  Tanimoto, N  Yamasaki, K  Nakanishi, S  Kitada, K  Makiyama, T  Akao, M  Kita, T  Sasa, M  Serikawa, T 
Citation: Gohma H, etal., Physiol Genomics. 2006 Feb 14;24(3):198-206. Epub 2005 Dec 20.
Pubmed: (View Article at PubMed) PMID:16368876
DOI: Full-text: DOI:10.1152/physiolgenomics.00221.2005

KCNQ1 forms K+ channels by assembly with regulatory subunit KCNE proteins and plays a key role in the K+ homeostasis in a variety of tissues. In the heart, KCNQ1 is coassembled with KCNE1 to produce a cardiac delayed rectifier K+ current. In the inner ear, the KCNQ1/KCNE1 complex maintains the high concentration of K+ in the endolymph. In the stomach, KCNQ1 is coassembled with KCNE2 to form the K+ exflux channel that is essential for gastric acid secretion. In the colon and small intestine, KCNQ1 is coassembled with KCNE3 to play an important role in transepithelial cAMP-stimulated Cl- secretion. For further understanding of Kcnq1 function in vivo, an animal model has been required. Here we reported the identification of a coisogenic Kcnq1 mutant rat, named deafness Kyoto (dfk), and the characterization of its phenotypes. WTC-dfk rats carried intragenic deletion at the Kcnq1 gene and showed impaired gain of weight, deafness, and imbalance resulting from the marked reduction of endolymph, prolonged QT interval in the electrocardiogram (ECG), and gastric achlorhydria associated with hypertrophic gastric mucosa. Surprisingly, WTC-dfk rats showed hypertension, which suggested that Kcnq1 might be involved in the regulation of blood pressure. These findings suggest that WTC-dfk rats could represent a powerful tool for studying the physiological functions of KCNQ1 and for the establishment of new therapeutic procedures for Kcnq1-related diseases.

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CRRD Object Information
CRRD ID: 1581602
Created: 2006-10-07
Species: All species
Last Modified: 2006-10-07
Status: ACTIVE



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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.