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. Author manuscript; available in PMC: 2017 May 1.
Published in final edited form as: Curr Opin Cardiol. 2016 May;31(3):266–274. doi: 10.1097/HCO.0000000000000277

Figure 1. Current iPSC-CM models of inherited errors in metabolism.

Figure 1

(A) A schematic view of the process to recapitulate metabolic heart disease in a dish, with steps to reprogram human peripheral blood mononuclear cells to iPSCs and then differentiate to cardiomyocytes. (B) Cellular metabolic processes in inborn errors of metabolism successfully recapitulated in iPSC-CMs with correlated in vitro phenotypes. Pompe disease (glycogen storage disorder Type II) showed glycogen accumulation in lysosomes. Danon disease (glycogen storage disorder Type IIb) showed defects in early autophagosome vacuoles, which lead to cellular hypertrophy. Barth Syndrome (mitochondrial disorder) showed impaired cardiolipin remodeling resulting in reduced mitochondrial supercomplex formation. Fabry disease (lysosomal storage disorder) showed increased glycosphingolipid accumulation in lysosomes.