cardiovascular system
• junctional sarcoplasmic reticulum cisternae are wider and more variable in size compared to wild-type controls
• the chain like line of condensed CASQ2 is missing and the sarcoplasmic reticulum lumen is either empty or contains electron dense material that is not clustered
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• develop bidirectional ventricular tachycardia due to environmental stress
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• spontaneous ventricular premature beats
|
• at 5 Hz pacing 56% of myocytes developed delayed afterdepolarization and 28% developed triggered activity compared to 0% of wild-type controls
• incubation with epinephrine and 5 Hz pacing induces triggered activity in 43% of cell that did not develop triggered activity with 5 Hz pacing alone
• at 1 Hz pacing in the presence of epinephrine 4 of 17 mice develop early afterdepolarization
• sarcoplasmic reticulum Ca content is reduced by 35% compared to wild-type controls
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muscle
• junctional sarcoplasmic reticulum cisternae are wider and more variable in size compared to wild-type controls
• the chain like line of condensed CASQ2 is missing and the sarcoplasmic reticulum lumen is either empty or contains electron dense material that is not clustered
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
catecholaminergic polymorphic ventricular tachycardia 2 | DOID:0060676 |
OMIM:611938 |
J:151367 |