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The LMNA-R482L mutation induces changes in C2C12 cluster composition and increases the expression of genes related to connective tissue development, oxidative stress, stress defense, and autophagy in a population-specific manner

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This review describes how the four currently approved drug classes target the complex pathobiology of PAH and will consider the distinct target molecules of each drug class, their modes of action, and review the pivotal clinical trial data supporting their use