Targeted disruption of the Kvlqtgene causes deafness and…基因的靶向破坏导致耳聋并.pdfVIP

Targeted disruption of the Kvlqt1 gene causes deafness and gastric hyperplasia in mice Maxwell P. Lee,1,2 Jason D. Ravenel, 1,3 Ren-Ju Hu,4 Lawrence R. Lustig,5 Gordon Tomaselli,2 Ronald D. Berger,2 Sheri A. Brandenburg,1,2 Tracy J. Litzi,1,2 Tracie E. Bunton,6 Charles Limb,5 Howard Francis,5 Melissa Gorelikow,5 4 7 8 9 Hua Gu, Kay Washington, Pedram Argani, James R. Goldenring, Robert J. Coffey,10 and Andrew P. Feinberg1,2,3 1Institute of Genetic Medicine, 2Department of Medicine, and 3Department of Molecular Biology and Genetics, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA 4Laboratory of Immunology, National Institute of Allergy and Infectious Diseases, Rockville, Maryland, USA 5Department of Otorhinolaryngology, and 6Department of Comparative Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA 7Department of Pathology, Vanderbilt University Medical Center, Nashville, Tennessee, USA 8Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA 9Institute of Molecular Medicine and Genetics, Medical College of Georgia and the Veterans Affairs Medical Center, Augusta, Georgia, USA 10Departments of Cell Biology and Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA Address correspondence to: Andrew P. Feinberg, Department of Medicine, Johns Hopkins University School of Medicine, 720 Rutland Avenue, Ross 1064, Baltimore, Maryland 21205, USA. Phone: (410) 614-3489; Fax: (410) 614-9819; E-mail: afeinberg@. Received for publication July 27, 2000, and accepted in revised form October 26, 2000. The KvLQT1 gene encodes a voltage-gated potassium channel. Mutations in KvLQT1 underlie the dominantly transmitted Ward-Romano long QT syndrome, which causes cardiac arrhythmia, and the recessively transmitted Jervell and L