Phenotypic Analysis of Vertigo 2 Jackson Mice with a Kcnq1 Potassium Channel Mutation

• Published in: Experimental Animals Vol. 56; no. 4; pp. 295 - 300
• Main Authors: TAKAGI, Takeshi, NISHIO, Hajime, YAGI, Takeo, KUWAHARA, Masayoshi, TSUBONE, Hirokazu, TANIGAWA, Nobuhiko, SUZUKI, Koichi
• Format: Journal Article
• Language: English
• Published: Japan Japanese Association for Laboratory Animal Science 01.07.2007
• Subjects: Achlorhydria - blood; Achlorhydria - genetics; Achlorhydria - pathology; adrenal system; Animals; Corticosterone - blood; Disease Models, Animal; Electrocardiography; Female; Gastric Acid - metabolism; Gastric Acidity Determination; Gastric Mucosa - metabolism; Gastrins - blood; Genotype; hypertension; Hypertension - genetics; Hypertension - physiopathology; KCNQ1 Potassium Channel - genetics; long QT syndrome; Long QT Syndrome - genetics; Long QT Syndrome - pathology; Long QT Syndrome - physiopathology; Male; Mice; Mice, Inbred C3H; Mice, Mutant Strains; Mutation; Phenotype; stomach; Stomach - pathology
• ISSN: 1341-1357; 1881-7122
• DOI: 10.1538/expanim.56.295

The KCNQ1 gene encodes a voltage-dependent potassium ion channel, and mutations in this gene are the most common cause of congenital long QT syndrome (LQTS). In the present study, we investigated the various phenotypic characteristics of vertigo 2 Jackson (C3H/HeJCrl-Kcnq1vtg-2J/J) mice with a Kcnq1 mutation. Both heterozygotes (vtg-2J/+) and homozygotes (vtg-2J/vtg-2J) showed prolonged QT intervals in electrocardiograms (ECGs) compared to C3H/HeJ control (+/+) mice. Furthermore, vtg-2J/vtg-2J mice showed gastric achlorhydria associated with elevation of their serum gastrin levels. The serum corticosterone levels were also significantly increased in vtg-2J/vtg-2J mice. In addition, vtg-2J/vtg-2J mice exhibited significantly higher blood pressure. These findings indicate that the Kcnq1 mutation in vtg-2J mice alters various physiological functions in the cardiac, gastric and adrenocortical systems, and suggest that vtg-2J mice may represent a useful model for studying Kcnq1 functions.