PRKAG2 Cardiac Syndrome is a rare familial disorder that affects the heart in several ways. Early recognition allows interventions that can prolong survival.
Chromosome: 7q36
Gene affected: PRKAG2
Gene product: gamma-2 regulatory subunit of AMP-activated kinase (AMPK)
Inheritance: autosomal dominant
Features may include:
(1) cardiac hypertrophy with glycogen storage cardiomyopathy
