Bone Morphogenetic Protein Receptor Type II Deficiency and Increased Inflammatory Cytokine Production. A Gateway to Pulmonary Arterial Hypertension

Elaine Soon; Alexi Crosby; Mark Southwood; Peiran Yang; Tamara Tajsic; Mark Toshner; Sarah Appleby; Catherine M. Shanahan; Kenneth D. Bloch; Joanna Pepke‐Żaba; Paul D. Upton; Nicholas W. Morrell

doi:10.1164/rccm.201408-1509oc

June 13, 2015American Journal of Respiratory and Critical Care MedicineOpen Access

Bone Morphogenetic Protein Receptor Type II Deficiency and Increased Inflammatory Cytokine Production. A Gateway to Pulmonary Arterial Hypertension

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Key Points

To determine whether BMPR-II deficiency primes pulmonary vascular cells for an exaggerated inflammatory response that precipitates pulmonary arterial hypertension.
Examined pulmonary artery smooth muscle cells isolated from Bmpr2(+/-) mice, human patients harboring BMPR2 mutations, and wild-type controls under lipopolysaccharide stimulation.
Challenged heterozygous Bmpr2(+/-) mice and wild-type littermates with acute and chronic lipopolysaccharide regimens in vivo, with a cohort coadministered the superoxide dismutase mimetic tempol.
Acute lipopolysaccharide challenge provoked significantly higher pulmonary and circulating IL-6 and KC/IL-8 levels in Bmpr2(+/-) mice and patient-derived smooth muscle cells compared with wild-type controls.
Deficiency of BMPR-II was accompanied by increased STAT3 phosphorylation and a concomitant loss of extracellular superoxide dismutase in both mouse and human pulmonary artery smooth muscle cells.
Chronic lipopolysaccharide induced pulmonary hypertension in Bmpr2(+/-) mice but not in wild-type littermates, while tempol coadministration reversed hyperinflammation and prevented disease onset.

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Implication

Preclinical study demonstrates that BMPR-II deficiency exacerbates inflammation-induced pulmonary hypertension in mouse models and human cells, suggesting a dual-hit mechanism for disease onset.

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Soon et al. (2015) studied this question.

synapsesocial.com/papers/6a1559570c3a39952e9f6728 https://doi.org/10.1164/rccm.201408-1509oc

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Bone Morphogenetic Protein Receptor Type II Deficiency and Increased Inflammatory Cytokine Production. A Gateway to Pulmonary Arterial Hypertension

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Bone Morphogenetic Protein Receptor Type II Deficiency and Increased Inflammatory Cytokine Production. A Gateway to Pulmonary Arterial Hypertension

Key Points

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Implication

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Cite This Study

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