Figure 1. Proposed pathogenesis model of LMNA cardiomyopathy. LMNA mutation activates AKT-MTOR and impairs autophagy. This impairment results in energy stress and/or accumulation of toxic proteins that ultimately leads to cell damage and cardiomyopathy. Temsirolimus inhibits MTOR and mitigates pathogenic effects of impaired autophagy. Dashed lines indicate hypothetical connections that require further studies to validate.
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