Article
Lysosomal dysfunction and inflammatory sterol metabolism in pulmonary arterial hypertension.
Science (New York, N.Y.) - 24 Jan 2025
Harvey Lloyd D, Alotaibi Mona, Tai Yi-Yin, Tang Ying, Kim Hee-Jung J, Kelly Neil J, Sun Wei, Woodcock Chen-Shan C, Arshad Sanya, Culley Miranda K, El Khoury Wadih, Xie Rong, Al Aaraj Yassmin, Zhao Jingsi, Hafeez Neha, Rao Rashmi J, Jiang Siyi, Negi Vinny, Kirillova Anna, Perk Dror, Watson Annie M, St Croix Claudette M, Stolz Donna B, Lee Ji Young, Cheng Mary Hongying, Zhang Manling, Detmer Samuel, Guzman Edward, Manan Rajith S, Saggar Rajan, Haley Kathleen J, Waxman Aaron B, Okawa Satoshi, Schwantes-An Tae-Hwi, Pauciulo Michael W, Wang Bing, Webb Amy, Chauvet Caroline, Anderson Daniel G, Nichols William C, Desai Ankit A, Lafyatis Robert, Nouraie S Mehdi, Wu Haodi, McDonald Jeffrey G, Cheng Susan, Bahar Ivet, Bertero Thomas, Benza Raymond L, Jain Mohit, Chan Stephen Y
Abstract excerpt
Vascular inflammation regulates endothelial pathophenotypes, particularly in pulmonary arterial hypertension (PAH). Dysregulated lysosomal activity and cholesterol metabolism activate pathogenic inflammation, but their relevance to PAH is unclear. Nuclear receptor coactivator 7 (NCOA7) deficiency in endothelium produced an oxysterol and bile acid signature through lysosomal dysregulation, promoting endothelial...
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