Table 1.

Summary of mitochondrial studies using iPSC-CMs.

Target	Product	Pathway	Disease	Reference
mtDNA	mtDNA deletions	mtDNA homeostasis	Kearns-Sayre syndrome	Sequiera et al., Cells (2021) [40]
mt-RNR2	16S mt-rRNA	DNA replication	Hypertrophic cardiomyopathy	Li et al., Stem CellReports (2018) [41]
POLG	POLG2	DNA replication	Leigh syndrome	Liang et al., EMBO Medicine (2020) [42]
mt-TL1	mt-tRNA	Translation	MELAS	Yokota et al., Cell Death and Disease (2017) [46]
mtDNA	mtDNA mutations	mtDNA homeostasis	MELAS	Perales-Clemente et al., EMBO (2016) [47]
TAZ	Tafazzin 1	Cardiolipin production	Barth syndrome	Wang et al., Nature Medicine (2014) [53]
DNAJC19	TIM44	Protein import	Dilated cardiomyopathy	Rohani et al., Canadian Journal of Cardiology (2020) [56]
HADHA	ECHA	Fatty acid oxidation	Sudden infant death syndrome	Miklas et al., Nature Communications (2019) [69]
FXN	Frataxin	Iron-sulfur cluster production	Friedreich’s ataxia	Hick et al., Disease Models and Mechanisms (2013) [74]
SCO2	SCO2	Complex IV Assembly	Cardiomyopathy	Hallas et al., J Mol. Cell Medicine (2018) [76]