a novel c-terminal truncation scn5a mutation from a patient with sick sinus syndrome, conduction...

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A novel C-terminal truncation SCN5A mutation from a patient with sick sinus syndrome, conduction disorder and ventricular tachycardia by Bi-Hua Tan, Pedro Iturralde-Torres, Argelia Medeiros-Domingo, Santiago Nava, David J. Tester, Carmen R. Valdivia, Teresa Tusié-Luna, Michael J. Ackerman, and Jonathan C. Makielski Cardiovasc Res Volume 76(3):409-417 February 1, 2008 Copyright © 2007, European Society of Cardiology

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(A) Topological diagram of SCN5A showing a 4 base pair deletion (TCTG) after codon at no Bi-Hua Tan et al. Cardiovasc Res 2007;76: Copyright © 2007, European Society of Cardiology

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Page 1: A novel C-terminal truncation SCN5A mutation from a patient with sick sinus syndrome, conduction disorder…

A novel C-terminal truncation SCN5A mutation from a patient with sick sinus syndrome, conduction disorder and

ventricular tachycardia

by Bi-Hua Tan, Pedro Iturralde-Torres, Argelia Medeiros-Domingo, Santiago Nava, David J. Tester, Carmen R. Valdivia, Teresa Tusié-Luna, Michael J. Ackerman, and

Jonathan C. Makielski

Cardiovasc ResVolume 76(3):409-417

February 1, 2008

Copyright © 2007, European Society of Cardiology

Page 2: A novel C-terminal truncation SCN5A mutation from a patient with sick sinus syndrome, conduction disorder…

Electrocardiographic phenotypes.

Bi-Hua Tan et al. Cardiovasc Res 2007;76:409-417

Copyright © 2007, European Society of Cardiology

Page 3: A novel C-terminal truncation SCN5A mutation from a patient with sick sinus syndrome, conduction disorder…

(A) Topological diagram of SCN5A showing a 4 base pair deletion (TCTG) after codon at no. 1821.

Bi-Hua Tan et al. Cardiovasc Res 2007;76:409-417

Copyright © 2007, European Society of Cardiology

Page 4: A novel C-terminal truncation SCN5A mutation from a patient with sick sinus syndrome, conduction disorder…

Comparing the peak or late current of mutant L1821fs/10 and WT in the most common splice variant SCN5A-Q1077del background.

Bi-Hua Tan et al. Cardiovasc Res 2007;76:409-417

Copyright © 2007, European Society of Cardiology

Page 5: A novel C-terminal truncation SCN5A mutation from a patient with sick sinus syndrome, conduction disorder…

Voltage dependence of time to peak (A), fast time constant (B), slow time constant (C), and fast fractional amplitude of current decay (D).

Bi-Hua Tan et al. Cardiovasc Res 2007;76:409-417

Copyright © 2007, European Society of Cardiology

Page 6: A novel C-terminal truncation SCN5A mutation from a patient with sick sinus syndrome, conduction disorder…

Voltage-dependent gating for L1821fs/10 and WT in the Q1077del splice variant background.

Bi-Hua Tan et al. Cardiovasc Res 2007;76:409-417

Copyright © 2007, European Society of Cardiology

Page 7: A novel C-terminal truncation SCN5A mutation from a patient with sick sinus syndrome, conduction disorder…

Confocal images of HEK-293 cells expressing the L1821fs/10 (top panel) and WT (bottom panel) with FLAG-tagged sodium channels.

Bi-Hua Tan et al. Cardiovasc Res 2007;76:409-417

Copyright © 2007, European Society of Cardiology