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Junctophilin-2 Regulates Mitochondrial Metabolism.

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    Biorxiv : the Preprint Server for Biology
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    Junctophilin-2 (JPH2) gene therapy improves right ventricular (RV) function by restoring mitochondrial health in right ventricular dysfunction (RVD). This approach offers a novel therapeutic strategy for RVD, enhancing RV contractility without worsening pulmonary artery hypertension.

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    Area of Science:

    • Cardiovascular Biology and Disease
    • Mitochondrial Biology and Metabolism
    • Gene Therapy and Regenerative Medicine

    Background:

    • Right ventricular dysfunction (RVD) is a significant risk factor for mortality in cardiovascular diseases, with limited therapeutic options.
    • Current treatments for left heart failure are largely ineffective for RVD, highlighting the need for novel strategies targeting RV function.
    • Junctophilin-2 (JPH2) is crucial for cardiomyocyte function, influencing calcium handling, t-tubule structure, and potentially mitochondrial regulation.

    Approach:

    • Investigated the role of Junctophilin-2 (JPH2) in cardiomyocyte mitochondrial function and its potential as a therapeutic target for RVD.
    • Examined JPH2's interaction with mitofusin-2 (MFN2) and its impact on mitochondrial biogenesis, oxidative capacity, and lipid handling in induced pluripotent stem cell-derived cardiomyocytes (iPSC-CM).
    • Utilized adeno-associated virus serotype 9 (AAV9) gene therapy to deliver JPH2 in a rat model of RVD induced by monocrotaline.

    Key Points:

    • JPH2 directly interacts with MFN2; JPH2 ablation impairs mitochondrial biogenesis, oxidative capacity, and lipid handling in iPSC-CM.
    • AAV9-mediated JPH2 gene therapy corrected RV mitochondrial morphology, regulated fatty acid metabolism enzymes, and restored the RV lipidomic profile in the RVD rat model.
    • AAV9-JPH2 treatment improved RV function, demonstrating an inotropic effect without exacerbating pulmonary arterial hypertension (PAH) severity.

    Conclusions:

    • JPH2 plays a critical role in maintaining RV mitochondrial function and lipid metabolism.
    • Gene therapy with AAV9-JPH2 represents a promising therapeutic strategy for RVD, improving RV contractility.
    • Targeting JPH2 offers a novel approach to enhance RV function in various cardiac conditions associated with RVD.