9/20/2023 0 Comments Konfuz pahIn pulmonary arterial hypertension (PAH), pathologic vascular remodeling distorts the gross- and micro-scale structure of the pulmonary arterial vasculature, severely disrupting blood flow patterns throughout the cardiopulmonary circulation. TGF-β Superfamily Dysregulation Is a Critical Component of PAH These strategies could potentially reverse pulmonary arterial remodeling in PAH by targeting causative mechanisms and therefore hold significant promise for the PAH patient population. We review the TGF-β superfamily mechanisms underlying PAH pathogenesis, superfamily interactions with inflammation and mechanobiological forces, and therapeutic strategies under development that aim to restore SMAD signaling balance in the diseased pulmonary arterial vessels. Imbalanced signaling by the transforming growth factor-β (TGF-β) superfamily contributes extensively to dysregulated vascular cell proliferation in PAH, with overactive pro-proliferative SMAD2/3 signaling occurring alongside deficient anti-proliferative SMAD1/5/8 signaling. Available treatments improve physical activity and slow disease progression, but they act primarily as vasodilators and have limited effects on the biological cause of the disease-the uncontrolled proliferation of vascular endothelial and smooth muscle cells. Pulmonary arterial hypertension (PAH) is a rare disease characterized by high blood pressure in the pulmonary circulation driven by pathological remodeling of distal pulmonary arteries, leading typically to death by right ventricular failure.
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