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Cardiomyopathy-Associated Gene 1-Sensitive PKC-Dependent Connexin 43 Expression and Phosphorylation in Left

Yuanyuan Xie, Shenghua Liu, Shengshou Hu

    Cellular Physiology and Biochemistry : International Journal of Experimental Cellular Physiology, Biochemistry, and Pharmacology
    |November 28, 2017
    PubMed

    Insights

    Cardiomyopathy-associated gene 1 (CMYA1) affects connexin expression and gap junction communication, impacting Left Ventricular Noncompaction Cardiomyopathy (LVNC) pathogenesis via the Protein Kinase C pathway.

    Area of Science:

    • Cardiovascular Biology
    • Molecular Cardiology
    • Genetic Research

    Background:

    • Cardiomyopathy-associated gene 1 (CMYA1) is crucial for cardiac development and repair.
    • Abnormal CMYA1 expression is linked to cardiac dysplasia and cardiomyopathy.
    • Left Ventricular Noncompaction Cardiomyopathy (LVNC) is a primary cardiomyopathy with unclear pathogenesis.

    Purpose of the Study:

    • To investigate the relationship between CMYA1 and LVNC pathogenesis.
    • To explore CMYA1's effects on connexins (Cx) and gap junction intercellular communication (GJIC).
    • To identify the signaling pathway involved in CMYA1's action.

    Main Methods:

    • Western blotting, RT-qPCR, immunohistochemistry, immunofluorescence, co-immunoprecipitation.
    • Scrape loading-dye transfer assay to assess GJIC.
    • Analysis in human normal and LVNC myocardial tissues and HL1 cells.

    Main Results:

    • CMYA1 expression inversely correlated with Cx43 and Cx40.
    • Increased and disordered CMYA1 expression observed at intercalated discs in LVNC tissues.
    • CMYA1 interacts with Cx43 and positively correlates with p-Cx43 (S368) via the Protein Kinase C (PKC) pathway.
    • Altered CMYA1 levels significantly affected GJIC in HL1 cells.

    Conclusions:

    • Abnormal CMYA1 expression impacts Cx43 expression and phosphorylation through the PKC signaling pathway.
    • CMYA1 plays a role in regulating GJIC.
    • CMYA1 is implicated in the molecular mechanisms underlying LVNC pathogenesis.
    Abstract

    Keywords:
    Cardiomyopathy-associated gene 1 (CMYA1)Connexin 43 (Cx43)Left ventricular noncompaction cardiomyopathy (LVNC)PKC signaling pathway

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