Thomas Bertero

Pulmonary Primary Oxysterol and Bile Acid Synthesis as a Predictor of Outcomes in Pulmonary Arterial Hypertension

Mona Alotaibi, Lloyd D. Harvey, William C. Nichols, Michael W. Pauciulo et autres

Pulmonary arterial hypertension (PAH) is a rare and fatal vascular disease with heterogeneous clinical manifestations. To date, molecular determinants underlying the development of PAH and related outcomes remain poorly understood. Herein, we identify pulmonary primary oxysterol and bile acid synthesis (PPOBAS) as …

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Endothelial ADAR1 Deficit Induces the NOCT-IRF7 Axis in Pulmonary Hypertension

Chen-Shan Chen Woodcock, Giovanni Maroli, Hyunbum Kim, Yi‐Yin Tai et autres

BACKGROUND: Early apoptosis of pulmonary artery endothelial cells (PAECs) is a driver of vascular remodeling and pulmonary hypertension (PH), but its regulation is poorly defined. Adenosine deaminase acting on RNA 1 (ADAR1, gene name ADAR ) is an RNA editing enzyme that …

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Deficiency of Smooth Muscle Adar1 Exacerbates Vascular Remodeling and Pulmonary Hypertension

Yunhye Kim, Giovanni Maroli, Chen-Shan Chen Woodcock, Hyunbum Kim et autres

BACKGROUND: ADAR1 (adenosine deaminase acting on RNA 1) catalyzes the conversion of adenosine to inosine in double stranded RNAs (dsRNAs), which is critical to prevent autoinflammatory responses mediated by activation of the type I IFN (interferon) signaling. We define the role of …

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Correction to: BOLA (BolA Family Member 3) Deficiency Controls Endothelial Metabolism and Glycine Homeostasis in Pulmonary Hypertension

Qiujun Yu, Yi-Yin Tai, Ying Tang, Jingsi Zhao et autres

Lysosomal dysfunction and inflammatory sterol metabolism in pulmonary arterial hypertension

Lloyd D. Harvey, Mona Alotaibi, Yi‐Yin Tai, Ying Tang et autres

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 …

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Mechano-dependent sorbitol accumulation supports biomolecular condensate

Stéphanie Torrino, William M. Oldham, Andrés R. Tejedor, Ignacio Sanchez‐Burgos et autres

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GLS2 links glutamine metabolism and atherosclerosis by remodeling artery walls

Florent Murcy, Coraline Borowczyk, Samuel Gourion‐Arsiquaud, Stéphanie Torrino et autres

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Dietary intake and glutamine-serine metabolism control pathologic vascular stiffness

Nesrine Rachedi, Ying Tang, Yi-Yin Tai, Jingsi Zhao et autres

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DHCR7 Deficiency Promotes 7β-hydroxycholesterol Accumulation and Endothelial Apoptosis in Pulmonary Hypertension

Rashmi Rao, Hee-Jung Kim, Shan Jiang, Yi‐Yin Tai et autres

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Genetic regulation and targeted reversal of lysosomal dysfunction and inflammatory sterol metabolism in pulmonary arterial hypertension

Lloyd D. Harvey, Mona Alotaibi, Hee-Jung Janice Kim, Yi‐Yin Tai et autres

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. In human pulmonary arterial ECs and in PAH, …

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Allele-specific control of rodent and human lncRNA KMT2E-AS1 promotes hypoxic endothelial pathology in pulmonary hypertension

Yi‐Yin Tai, Qiujun Yu, Ying Margie Tang, Wei Sun et autres

Hypoxic reprogramming of vasculature relies on genetic, epigenetic, and metabolic circuitry, but the control points are unknown. In pulmonary arterial hypertension (PAH), a disease driven by hypoxia inducible factor (HIF)–dependent vascular dysfunction, HIF-2α promoted expression of neighboring genes, long noncoding RNA (lncRNA) …

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Abstract 17196: Genetic and Acquired DHCR7 Deficiency Upregulates 7β-hydroxycholesterol to Drive Endothelial Apoptosis and Pulmonary Hypertension

Rashmi J. Rao, Heejung Kim, Siyi Jiang, Yi‐Yin Tai et autres

Introduction: Pulmonary hypertension (PH) is a fatal disease without a cure, in which endothelial dysfunction drives pathologic remodeling of the pulmonary vasculature. Individuals with Smith-Lemli-Opitz syndrome (SLOS) develop PH, but the underlying mechanisms remain undefined. SLOS is an autosomal recessive disorder of …

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