BMPR2 mutations are the most common genetic cause of pulmonary arterial hypertension (PAH). Pulmonary artery endothelial cells (PAECs) with reduced BMPR2 expression are linked to a persistent DNA damage after reoxygenation. Forkhead box F1 (FOXF1) is a transcription factor with affinity for endothelial cells in the lung, and its reduced expression has also been associated with DNA damage in those cells and PAH.
Chiesi Farmaceutici SpA has disclosed imidazole derivatives acting as TGF-β receptor type-1 (TGFBR1; ALK5; SKR4; TβR-I) inhibitors reported to be useful for the treatment of idiopathic pulmonary fibrosis (IPF).
Based on previous studies that have demonstrated the potential of growth hormone-releasing hormone receptor (GHRH-R) antagonists to modulate immune responses to bleomycin lung injury, researchers from the University of Miami and affiliated organizations aimed to evaluate the potential of the GHRH-R antagonist MIA-602 in a mouse model of rVSV-SARS-CoV-2-induced pulmonary dysfunction and heart injury.
ΔF508-CFTR is the most common CFTR mutation in cystic fibrosis (CF), which leads to destabilization of CFTR’s first nucleotide-binding domain (NBD1), contributing centrally to defective ΔF508-CFTR folding, trafficking and function.
Researchers from Arcturus Therapeutics Inc. and affiliated organizations presented preclinical data for LUNAR-CFTR, a novel mutation-agnostic, aerosolized CFTR replacement therapy for the treatment of cystic fibrosis (CF).
Rapt Therapeutics Inc. has described chemokine CCR4 receptor antagonists reported to be useful for the treatment of cancer, allergic asthma, contact dermatitis, allergic rhinitis, immunological, inflammatory, cardiovascular and metabolic disorders.
Vertex Pharmaceuticals Inc. has identified cystic fibrosis transmembrane conductance regulator (CFTR) modulators reported to be useful for the treatment of cystic fibrosis.
Work at the Agency For Science Technology & Research Bioprocessing Technology Institute has led to the identification of mitogen-activated protein kinase kinase kinase 14 (MAP3K14; NIK) inhibitors reported to be useful for the treatment of pulmonary fibrosis.
Chiesi Farmaceutici SpA has described amido cyclopropyl derivatives acting as lysophospholipid LPA1 receptor (LPAR1; EDG2) antagonists reported to be useful for the treatment of idiopathic pulmonary fibrosis (IPF).
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