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Printable Handouts
Navigable Slide Index
- Introduction
- Cellular action potentials and the ECG
- The long QT syndrome
- Ion channels - depolarization & repolarization
- Balances of outward & inward currents in APD
- Two critical potassium channels in the heart
- LQTS: genetic linkage to ion channel genes
- Molecular architecture of the IKS channel
- What triggers fatal events?
- IKS signaling complex and long QT syndrome
- IKS channel and response to beta-AR regulation
- The IKS alpha subunit is posphorylated by PKA
- Transgenic hKCNQ1_KCNE1 mouse
- Functional regulation
- Inherited KCNQ1 mutation disrupts the complex
- KCNQ1 mutations and IKS regulation
- Mutations in Yotiao are also linked to LQTS
- Point mutation S1570L in Yotiao binding domain
- Yotiao C-T: a hot spot?
- IKS macromolecular complex
- Disruption of IKS signaling microdomain in LQT-1
- KCNQ1 hyperphosphorylation
- Two KCNQ1 mutations on adjacent residues
- Both atrial fibrilation mutations slow deactivation
- Probing the location of KCNE1 in the IKS channel
- Spontaneous crosslinking: KCNE1/KCNQ1
- E1 location within KCNQ1/KCNE1 channel
- Distinct orientation of the 2 AF mutant residues
- Predicted proximity of KCNE1 & KCNQ1 V141
- How does KCNE1 control KCNQ1 gating?
- psKCNQ1 Alexa binding site introduced
- psKCNQ1: voltage sensor to gate coupling
- KCNE1 alters voltage sensor movement
- KCNE1/KCNQ1 in the assembled IKS channel
- Implications of KCNE1/KCNQ1 stoichiometry
- Subunit stoichiometry
- Summary
- 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
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 November 4, 2024, from https://doi.org/10.69645/GJBP2748.Export Citation (RIS)
Publication History
Financial Disclosures
- Prof. Robert Kass has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.
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