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Michael W Pauciulo

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Articles 60
Citations 1232
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Recent Articles
1.
Harvey L, Alotaibi M, Tai Y, Tang Y, Kim H, Kelly N, et al.
Science . 2025 Jan; 387(6732):eadn7277. PMID: 39847635
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...
2.
Gomez-Arroyo J, Houweling A, Bogaard H, Aman J, Kitzmiller J, Porollo A, et al.
bioRxiv . 2024 Sep; PMID: 39345371
Rationale: Approximately 80% of patients with non-familial pulmonary arterial hypertension (PAH) lack identifiable pathogenic genetic variants. While most genetic studies of PAH have focused on predicted loss-of-function variants, recent approaches...
3.
Hong J, Medzikovic L, Sun W, Wong B, Ruffenach G, Rhodes C, et al.
Circulation . 2024 Aug; 150(16):1268-1287. PMID: 39167456
Background: Integrative multiomics can elucidate pulmonary arterial hypertension (PAH) pathobiology, but procuring human PAH lung samples is rare. Methods: We leveraged transcriptomic profiling and deep phenotyping of the largest multicenter...
4.
Griffiths M, Simpson C, Yang J, Vaidya D, Nies M, Brandal S, et al.
Chest . 2024 Aug; 166(6):1511-1531. PMID: 39154795
Background: Risk assessment in pulmonary arterial hypertension (PAH) is fundamental to guiding treatment and improved outcomes. Clinical models are excellent at identifying high-risk patients, but leave uncertainty amongst moderate-risk patients....
5.
Gao L, Skinner J, Nath T, Lin Q, Griffiths M, Damico R, et al.
Respir Res . 2024 Jun; 25(1):235. PMID: 38844967
Background: Abnormal remodeling of distal pulmonary arteries in patients with pulmonary arterial hypertension (PAH) leads to progressively increased pulmonary vascular resistance, followed by right ventricular hypertrophy and failure. Despite considerable...
6.
Tang H, Gupta A, Morrisroe S, Bao C, Schwantes-An T, Gupta G, et al.
Circulation . 2024 May; 150(4):302-316. PMID: 38695173
Background: The ubiquitin-proteasome system regulates protein degradation and the development of pulmonary arterial hypertension (PAH), but knowledge about the role of deubiquitinating enzymes in this process is limited. UCHL1 (ubiquitin...
7.
Harvey L, Alotaibi M, Kim H, Tai Y, Tang Y, Sun W, et al.
bioRxiv . 2024 Mar; PMID: 38464060
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...
8.
Alotaibi M, Harvey L, Nichols W, Pauciulo M, Hemnes A, Long T, et al.
bioRxiv . 2024 Feb; PMID: 38328113
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....
9.
Al-Qazazi R, Emon I, Potus F, Martin A, Lima P, Vlasschaert C, et al.
medRxiv . 2024 Jan; PMID: 38234783
Background: Mutations are found in 10-20% of idiopathic PAH (IPAH) patients, but none are consistently identified in connective tissue disease-associated PAH (APAH), which accounts for ∼45% of PAH cases. mutations,...
10.
Tai Y, Yu Q, Tang Y, Sun W, Kelly N, Okawa S, et al.
Sci Transl Med . 2024 Jan; 16(729):eadd2029. PMID: 38198571
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...