Tables Table 1 Pharmacological testing in adult CASQ2^△/△mice

Agent and Dose	Mechanism of Action	No. mice	Sinus Rate eats/min	Rest			Exercise			Epinephrine			mice with VT
				VPC s	NSV T	SV T	VPC s	NSV T	SV T	VPC s	NSV T	SVT
Saline 0.1 ml	Control	6	657±22	6	6	0	6	6	4	6	6	5	6
Reserpine acute 0.5mg/g	Neurotransmitter depletion	3	648±10	3	2	0	3	3	1	3	3	3	3
Reserpine – 0.5mg/g	Neurotransmitter depletion	3	637± 6	3	2	0	3	3	2			2	3
Propranolol 10mg/g	β1+pβ2 Blocker	6	456±31¶	3	1	0	6	3	0	6	5	0	5
Metoprolol 10mg/g	β1 Blocker	6	449±11¶	2	0	0	6	5	0	0	0	0	5
Phentolamine 50 mg/g	α Blocker	6	510±45 *	0	0	0	1	0	0	0	0	0	0†
Labetalol 20 mg/g	β1+β2+α1 Blocker	7	409± 6¶	1	0	0	1	0	0	0	0	0	0†
Labetalol 10 mg/g	β1+β2+α1 Blocker	6	460±42¶	2	0	0	4	2	0	0	0	0	2*

Abbreviation: VPCs, ventricular premature contractions; NSVT, non-sutained ventricular tachycardia; SVT, sustained ventricular tachycardia; Sinus rate (beats/min); mice with VT, a criterion for disease expression defined as ventricular tachycardia under any condition;

^*P<0.05

^†P<0.01

^¶P<0.005. The sinus rate was determined at rest.