Membrane Biology & Protein Trafficking
|
|
Faculty
|
| David Andes |
| Sebastian Bednarek |
| Elizabeth Craig |
| Loren Denlinger |
| Timothy Donohue |
| Yoshihiro Kawaoka |
| Deane Mosher |
| Marisa Otegui |
| Ronald Raines |
| Linda Schuler |
| Ahna Skop |
| Edgar Spalding |
| Jeff Walker |
| John White |
|
Long QT syndrome (LQTS) is an inherited or acquired disease associated with episodic ventricular arrhythmias and sudden death. One form of inherited LQTS (LQTS-2) results from mutations in the human Ether-a-go-go-Related Gene 1 (hERG1, or KCNH2). hERG1 encodes a potassium channel with biophysical and pharmacological properties similar to those of cardiac IKr, thus explaining the underlying cause of LQTS-2 as a defect in this repolarizing current. Shown in upper left and lower right panels are 3D reconstructed images of immunohisto-chemically stained canine ventricular myocytes obtained from a Zeiss Apotome imaging system. hERG1 signal, seen in purple, occurs as regularly spaced punctate columns extending from the cell surface to the interior. Green signal, from Myosin binding protein C, appears as doublets delineating the sarcomere A-bands. hERG signal appears in the I-bands, adjacent to the A-bands. These data show hERG1 localizes to transverse tubules, where it is optimally positioned to shape the repolarizing phase of the cardiac action potential. |
About Membrane Biology & Protein Trafficking