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1. Introduction
2. Cellular action potentials and the ECG
3. The long QT syndrome
4. Ion channels - depolarization & repolarization
5. Balances of outward & inward currents in APD
6. Two critical potassium channels in the heart
7. LQTS: genetic linkage to ion channel genes
8. Molecular architecture of the IKS channel
9. What triggers fatal events?
10. IKS signaling complex and long QT syndrome
11. IKS channel and response to beta-AR regulation
12. The IKS alpha subunit is posphorylated by PKA
13. Transgenic hKCNQ1_KCNE1 mouse
14. Functional regulation
15. Inherited KCNQ1 mutation disrupts the complex
16. KCNQ1 mutations and IKS regulation
17. Mutations in Yotiao are also linked to LQTS
18. Point mutation S1570L in Yotiao binding domain
19. Yotiao C-T: a hot spot?
20. IKS macromolecular complex
21. Disruption of IKS signaling microdomain in LQT-1
22. KCNQ1 hyperphosphorylation
23. Two KCNQ1 mutations on adjacent residues
24. Both atrial fibrilation mutations slow deactivation
25. Probing the location of KCNE1 in the IKS channel
26. Spontaneous crosslinking: KCNE1/KCNQ1
27. E1 location within KCNQ1/KCNE1 channel
28. Distinct orientation of the 2 AF mutant residues
29. Predicted proximity of KCNE1 & KCNQ1 V141
30. How does KCNE1 control KCNQ1 gating?
31. psKCNQ1 Alexa binding site introduced
32. psKCNQ1: voltage sensor to gate coupling
33. KCNE1 alters voltage sensor movement
34. KCNE1/KCNQ1 in the assembled IKS channel
35. Implications of KCNE1/KCNQ1 stoichiometry
36. Subunit stoichiometry
37. Summary
38. Collaborators

Topics Covered

• The long QT syndrome
• LQTS: genetic linkage to ion channel genes
• Molecular IKS signaling complex and long QT syndrome
• IKS subunits: KCNQ1 and KCNE1
• KCNQ1 mutations and IKS regulation
• Yotiao: an additional component
• Mutations in Yotiao linked to LQTS
• IKS and other cardiac disorders
• The location of KCNE1 in the IKS channel
• Control of KCNQ1 gating by KCNE1
• Alteration of voltage sensor movement by KCNE1
• KCNE1/KCNQ1 stoichiometry and its implications

Links

Series:

• Ion Channels

Categories:

• Biochemistry
• Cell Biology

Talk Citation

Kass, R. (2011, April 10). Heritable arrhythmias: insight into molecular physiology from the long QT syndrome [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved April 15, 2025, from https://doi.org/10.69645/GJBP2748.
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Publication History

• Published on April 10, 2011

Financial Disclosures

• Prof. Robert Kass has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.