Increased BMP-Responsive Transcription Factors in Distinct Endothelial and Mesenchymal Cells in PAH

Overview

Journal Am J Respir Crit Care Med

Specialty Critical Care

Date 2024 Nov 5

PMID 39499865

Authors

Adam Michael Andruska

Rodrigo Cantu Valadez

Kenzo Ichimura

Pauline Chu

Tianyi Zhang

Katharina Schimmel

Lingli Wang

Aiqin Cao

Micheala A Aldred

Edda Spiekerkoetter

Affiliations

Soon will be listed here.

References

Zhu N, Gonzaga-Jauregui C, Welch C, Ma L, Qi H, King A . Exome Sequencing in Children With Pulmonary Arterial Hypertension Demonstrates Differences Compared With Adults. Circ Genom Precis Med. 2018; 11(4):e001887. PMC: 5896781. DOI: 10.1161/CIRCGEN.117.001887. View

Travaglini K, Nabhan A, Penland L, Sinha R, Gillich A, Sit R . A molecular cell atlas of the human lung from single-cell RNA sequencing. Nature. 2020; 587(7835):619-625. PMC: 7704697. DOI: 10.1038/s41586-020-2922-4. View

Yang J, Li X, Li Y, Southwood M, Ye L, Long L . Id proteins are critical downstream effectors of BMP signaling in human pulmonary arterial smooth muscle cells. Am J Physiol Lung Cell Mol Physiol. 2013; 305(4):L312-21. PMC: 3891012. DOI: 10.1152/ajplung.00054.2013. View

Tuder R, Gandjeva A, Williams S, Kumar S, Kheyfets V, Hatton-Jones K . Digital Spatial Profiling Identifies Distinct Molecular Signatures of Vascular Lesions in Pulmonary Arterial Hypertension. Am J Respir Crit Care Med. 2024; 210(3):329-342. PMC: 11348978. DOI: 10.1164/rccm.202307-1310OC. View

Atkinson C, Stewart S, Upton P, Machado R, Thomson J, Trembath R . Primary pulmonary hypertension is associated with reduced pulmonary vascular expression of type II bone morphogenetic protein receptor. Circulation. 2002; 105(14):1672-8. DOI: 10.1161/01.cir.0000012754.72951.3d. View

Lane K, Machado R, Pauciulo M, Thomson J, Phillips 3rd J, Loyd J . Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. Nat Genet. 2000; 26(1):81-4. DOI: 10.1038/79226. View

Deng Z, Morse J, Slager S, Cuervo N, Moore K, Venetos G . Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet. 2000; 67(3):737-44. PMC: 1287532. DOI: 10.1086/303059. View

Spiekerkoetter E, Tian X, Cai J, Hopper R, Sudheendra D, Li C . FK506 activates BMPR2, rescues endothelial dysfunction, and reverses pulmonary hypertension. J Clin Invest. 2013; 123(8):3600-13. PMC: 3726153. DOI: 10.1172/JCI65592. View

Korchynskyi O, Ten Dijke P . Identification and functional characterization of distinct critically important bone morphogenetic protein-specific response elements in the Id1 promoter. J Biol Chem. 2001; 277(7):4883-91. DOI: 10.1074/jbc.M111023200. View

10.

Orriols M, Gomez-Puerto M, Ten Dijke P . BMP type II receptor as a therapeutic target in pulmonary arterial hypertension. Cell Mol Life Sci. 2017; 74(16):2979-2995. PMC: 5501910. DOI: 10.1007/s00018-017-2510-4. View

11.

Westoo C, Norvik C, Peruzzi N, van der Have O, Lovric G, Jeremiasen I . Distinct types of plexiform lesions identified by synchrotron-based phase-contrast micro-CT. Am J Physiol Lung Cell Mol Physiol. 2021; 321(1):L17-L28. PMC: 8321861. DOI: 10.1152/ajplung.00432.2020. View

12.

Wang F, Flanagan J, Su N, Wang L, Bui S, Nielson A . RNAscope: a novel in situ RNA analysis platform for formalin-fixed, paraffin-embedded tissues. J Mol Diagn. 2011; 14(1):22-9. PMC: 3338343. DOI: 10.1016/j.jmoldx.2011.08.002. View

13.

Humbert M, McLaughlin V, Gibbs J, Gomberg-Maitland M, Hoeper M, Preston I . Sotatercept for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med. 2021; 384(13):1204-1215. DOI: 10.1056/NEJMoa2024277. View