Maintenance of Energy Metabolism Is an Integral Part of Plakophilin-2 and Desmosome Functions.

First, nontargeted polar metabolomics showed a trend toward reduced long-chain acylcarnitines, palmitoylcarnitine, oleoylcarnitine, and stearoylcarnitine in iPSC-CMs in response to acute silencing of PKP2 ( Figure 3B ).

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Perturbed energy metabolism of the ARVC mouse model, particularly impaired FAO and increased de novo fatty acid synthesis, was further investigated by shotgun lipidomics 49 , 50 to show an increasing trend in TAG levels, which were normalized in TN-401–treated cKO heart ( Figure 2D ).

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