. Author manuscript; available in PMC: 2013 Aug 1.

Published in final edited form as: Pediatr Cardiol. 2012 Mar 30;33(6):943–949. doi: 10.1007/s00246-012-0303-y

Table 2.

Representative mouse models of SCN5A related LQT3 syndrome.

Model	Locus of the mutation	Genetic approach	Biophysical consequences	References
WT/Δ1505–1507KPQ	intracellular loop between DIII and DIV of Nav1.5	knock-in	↑late I_Na,	Ref [13, 39, 40]
WT/N1325S	intracellular region between S4–S5 of DIII of the Nav1.5	transgenic	↑late I_Na	Ref [51]
WT/1798insD	C-terminal domain	knock-in	↑late I_Na, ↓I_Na	Ref [42]