DUSP5-mediated inhibition of smooth muscle cell proliferation suppresses pulmonary hypertension and right ventricular hypertrophy

Bradley S. Ferguson, Sara A. Wennersten, Kimberly M. Demos-Davies, Marcello Rubino, Emma L. Robinson, Maria A. Cavasin, Matthew S. Stratton, Andrew M. Kidger, Tianjing Hu, Stephen M. Keyse, Robert A. McKnight, Robert H. Lane, Eva S. Nozik, Mary C. M. Weiser-Evans, Timothy A. McKinsey (Lead / Corresponding author)

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    8 Citations (Scopus)
    80 Downloads (Pure)

    Abstract

    Pulmonary hypertension (PH) is associated with structural remodeling of pulmonary arteries (PAs) because of excessive proliferation of fibroblasts, endothelial cells, and smooth muscle cells (SMCs). The peptide hormone angiotensin II (ANG II) contributes to pulmonary vascular remodeling, in part, through its ability to trigger extracellular signal-regulated kinase (ERK1/2) activation. Here, we demonstrate that the ERK1/2 phosphatase, dual-specificity phosphatase 5 (DUSP5), functions as a negative regulator of ANG II-mediated SMC proliferation and PH. In contrast to wild-type controls, Dusp5 null mice infused with ANG II developed PH and right ventricular (RV) hypertrophy. PH in Dusp5 null mice was associated with thickening of the medial layer of small PAs, suggesting an in vivo role for DUSP5 as a negative regulator of ANG II-dependent SMC proliferation. Consistent with this, overexpression of DUSP5 blocked ANG II-mediated proliferation of cultured human pulmonary artery SMCs (hPASMCs) derived from patients with idiopathic PH or from failed donor controls. Collectively, the data support a role for DUSP5 as a feedback inhibitor of ANG II-mediated ERK signaling and PASMC proliferation and suggest that disruption of this circuit leads to adverse cardiopulmonary remodeling. NEW & NOTEWORTHY Dual-specificity phosphatases (DUSPs) serve critical roles in the regulation of mitogen-activated protein kinases, but their functions in the cardiovascular system remain poorly defined. Here, we provide evidence that DUSP5, which resides in the nucleus and specifically dephosphorylates extracellular signal-regulated kinase (ERK1/2), blocks pulmonary vascular smooth muscle cell proliferation. In response to angiotensin II infusion, mice lacking DUSP5 develop pulmonary hypertension and right ventricular cardiac hypertrophy. These findings illustrate DUSP5-mediated suppression of ERK signaling in the lungs as a protective mechanism.

    Original languageEnglish
    Pages (from-to)H382–H389
    Number of pages8
    JournalAmerican Journal of Physiology - Heart and Circulatory Physiology (AJP - Heart and Circulatory Physiology)
    Volume321
    Issue number2
    Early online date18 Jun 2021
    DOIs
    Publication statusPublished - Aug 2021

    Keywords

    • Angiotensin
    • ERK
    • Kinase
    • Pulmonary hypertension
    • Smooth muscle cell

    ASJC Scopus subject areas

    • Physiology
    • Cardiology and Cardiovascular Medicine
    • Physiology (medical)

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