Table 2.

Disease modeling utilizing hiPSC-CM, restricted to studies describing changes in energy metabolism.

Condition	Reported metabolic abnormalities in diseased hiPSC-CM	Ref.
Barth syndrome	Fragmented mitochondria, elevated basal oxygen consumption rates and impaired electron transport chain	[65] [124]
Pompe disease	Degenerated mitochondria, lower oxygen consumption rate, accumulation of glycogen	[[125], [126], [127]]
Friedreich's ataxia	mtDNA depletion, mitochondria network disorganization and lower level of respiratory chain proteins	[128]
Arrhythmogenic right ventricular dysplasia (ARVD)	PPARγ over-activation and lipid accumulation; diseased hiPSC-CM directly transdifferentiate into adipocytes	[66] [84] [[129], [130], [131]]
Danon's disease	Reduced mitophagic flux resulted in mitochondrial fragmentation	[132] [134]
Hypertrophic cardiomyopathy	Abnormally high metabolic respiration rate	[44]