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Lysosomal dysfunction and inflammatory sterol metabolism in pulmonary arterial hypertension
Editor’s summary Pulmonary arterial hypertension (PAH) is a poorly understood condition that remains difficult to treat and carries a high risk of mortality. Harvey et al. identified a nuclear receptor called NCOA7 that acts as a biological brake on inflammation in the lining of blood vessels (see the Perspective by Pullamsetti and Savai). In cells and in mouse and rat models, deficiency of NCOA7 promoted lysosomal dysfunction and the generation of proinflammatory bile acids and oxysterols.
Genetic regulation and targeted reversal of lysosomal dysfunction and inflammatory sterol metabolism in pulmonary arterial hypertension
Abstract Vascular inflammation critically regulates endothelial cell (EC) pathophenotypes, particularly in pulmonary arterial hypertension (PAH). Dysregulation of lysosomal activity and cholesterol metabolism have known inflammatory roles in disease, but their relevance to PAH is unclear.
Allele-specific control of rodent and human lncRNA KMT2E-AS1 promotes hypoxic endothelial pathology in pulmonary hypertension
Research Article PULMONARY HYPERTENSION Yi-Yin Tai https://orcid.org/0000-0003-3674-0783, Qiujun Yu https://orcid.org/0000-0003-0690-138X, [...] , Ying Tang, Wei Sun https://orcid.org/0000-0002-5255-1213, [...] , Neil J.
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