title: Differential expression of KvLQT1 and its regulator IsK in mouse epithelia.
abstract: KCNQ1 is the human gene responsible in most cases for the long QT syndrome, a genetic disorder characterized by anomalies in cardiac repolarization leading to arrhythmias and sudden death. KCNQ1 encodes a pore-forming K+ channel subunit termed KvLQT1 which, in association with its regulatory beta-subunit IsK (also called minK), produces the slow component of the delayed-rectifier cardiac K+ current. We used in situ hybridization to localize KvLQT1 and IsK mRNAs in various tissues from adult mice. We showed that KvLQT1 mRNA expression is widely distributed in epithelial tissues, in the absence (small intestine, lung, liver, thymus) or presence (kidney, stomach, exocrine pancreas) of its regulator IsK. In the kidney and the stomach, however, the expression patterns of KvLQT1 and IsK do not coincide. In many tissues, in situ data obtained with the IsK probe coincide with beta-galactosidase expression in IsK-deficient mice in which the bacterial lacZ gene has been substituted for the IsK coding region. Because expression of KvLQT1 in the presence or absence of its regulator generates a K+ current with different biophysical characteristics, the role of KvLQT1 in epithelial cells may vary depending on the expression of its regulator IsK. The high level of KvLQT1 expression in epithelial tissues is consistent with its potential role in K+ secretion and recycling, in maintaining the resting potential, and in regulating Cl- secretion and/or Na+ absorption.
authors.affiliation: Experimental and Molecular Cardiology Group, Academic Medical Center, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.
authors.forename: S
authors.initials: S
authors.lastname: Demolombe
authors.forename: D
authors.initials: D
authors.lastname: Franco
authors.forename: P
authors.initials: P
authors.lastname: de Boer
authors.forename: S
authors.initials: S
authors.lastname: Kuperschmidt
authors.forename: D
authors.initials: D
authors.lastname: Roden
authors.forename: Y
authors.initials: Y
authors.lastname: Pereon
authors.forename: A
authors.initials: A
authors.lastname: Jarry
authors.forename: A F
authors.initials: AF
authors.lastname: Moorman
authors.forename: D
authors.initials: D
authors.lastname: Escande
mesh_terms: D000818:Animals; D004622:Embryo, Mammalian; D004848:Epithelium / Q000378:metabolism; D007413:Intestinal Mucosa / Q000378:metabolism; D051656:KCNQ Potassium Channels; D051657:KCNQ1 Potassium Channel; D007668:Kidney / Q000378:metabolism*; D008168:Lung / Q000378:metabolism; D051379:Mice; D018482:Muscle, Skeletal / Q000378:metabolism; D009928:Organ Specificity / Q000502:physiology; D015221:Potassium Channels / Q000378:metabolism*; D024642:Potassium Channels, Voltage-Gated; D012333:RNA, Messenger / Q000378:metabolism
chemical_list: D051656:KCNQ Potassium Channels; D051657:KCNQ1 Potassium Channel; C496910:Kcnq1 protein, mouse; D015221:Potassium Channels; D024642:Potassium Channels, Voltage-Gated; D012333:RNA, Messenger; C059983:potassium channel protein I(sk)