Mouse Genome Informatics
ht
    Scn5atm1Agrc/Scn5a+
involves: 129/Sv
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
cardiovascular system
• old but not young mutants show extensive fibrosis of the ventricular myocardium (in the left and right ventricular free walls and in the interventricular septum)
• although young mutants do not exhibit ventricular fibrosis, they do show increased perivascular fibrosis which becomes massive with age
• show slight but significant bradycardia
• mutants exhibit a higher incidence of ventricular tachycardia than wild-type (25% vs. 6%)
• ventricular tachycardias begin at earlier times in the right ventricular outflow tract than at the base of the left ventricle
• mutant hearts exhibit abnormal electrophysiological properties of the right ventricle at the right ventricle outflow tract, showing increased heterogeneities in action potential duration, ventricular effective refractory periods, electrogram duration ratios and response latencies that correlate with an increased incidence of discordant alternans and with steeper restitution slopes
• PR intervals progressively prolong with age (from 3 to 71 weeks)
• P-wave intervals progressively prolong with age (from 3 to 71 weeks)
• QRS intervals progressively prolong with age (from 3 to 71 weeks)
• QTrc and QTc intervals are prolonged due to prolongation of the QRS interval

homeostasis/metabolism
• old but not young mutants show extensive fibrosis of the ventricular myocardium (in the left and right ventricular free walls and in the interventricular septum)
• although young mutants do not exhibit ventricular fibrosis, they do show increased perivascular fibrosis which becomes massive with age

Mouse Models of Human Disease
OMIM IDRef(s)
Brugada Syndrome 1; BRGDA1 601144 J:166438
Progressive Familial Heart Block, Type IA; PFHB1A 113900 J:109689