An adult patient with pulmonary arterial hypertension, a NOTCH3 mutation, and leflunomide exposure DOI Creative Commons

Elizabeth G. Fenner,

Catherine E. Simpson

Pulmonary Circulation, Год журнала: 2024, Номер 14(3)

Опубликована: Июль 1, 2024

Abstract Pulmonary arterial hypertension (PAH) is a poorly understood disease of the small pulmonary arteries. vascular remodeling and progressively rising resistance are hallmarks that ultimately result in right heart failure. Several genetic mutations, most notably bone morphogenetic protein receptor type 2, have causal association with heritable forms PAH. Mutations neurogenic locus notch homolog 3 ( NOTCH3) been reported adults children PAH, but whether NOTCH3 causally associated PAH debated. With this case report, we describe clinical characteristics, comorbidities, exposure history an adult patient multiple sclerosis who was found to missense mutation leflunomide.

Язык: Английский

Ginkgolide B Blocks Vascular Remodeling after Vascular Injury via Regulating Tgfβ1/Smad Signaling Pathway DOI Creative Commons
Quan Wang,

Shuai Ni,

Ling Li

и другие.

Cardiovascular Therapeutics, Год журнала: 2023, Номер 2023, С. 1 - 13

Опубликована: Дек. 13, 2023

Coronary artery disease (CAD) is the most prevalent cardiovascular worldwide, resulting in myocardial infarction (MI) and even sudden death. Following percutaneous coronary intervention (PCI), restenosis caused by vascular remodeling always formed at stent implantation site. Here, we show that Ginkgolide B (GB), a naturally occurring terpene lactone, effectively suppresses subsequent wild-type mice following left carotid (LCA) injury. Additional experiments reveal GB exerts protective effect on further through modulation of Tgfβ1/Smad signaling pathway vivo human smooth muscle cells (HVSMAs) but not umbilical vein endothelial (HUVECs) vitro. Moreover, beneficial abolished after incubated with pirfenidone (PFD, drug for idiopathic pulmonary fibrosis, IPF), which can inhibit Tgfβ1. In Tgfβ1-/- mice, treatment capsules Yinxingneizhi Zhusheye (including B) fails to improve restenosis. conclusion, our data identify could be potential novel therapeutic agent block vessel injury-associated significant repression pathway.

Язык: Английский

Процитировано

6

NOTCH3 and Pulmonary Arterial Hypertension DOI Open Access
Nolan M. Winicki, Cristian Puerta,

Casandra E. Besse

и другие.

International Journal of Molecular Sciences, Год журнала: 2024, Номер 25(11), С. 6248 - 6248

Опубликована: Июнь 6, 2024

NOTCH3 receptor signaling has been linked to the regulation of smooth muscle cell proliferation and maintenance cells in an undifferentiated state. Pulmonary arterial hypertension (World Health Organization Group 1 idiopathic disease: PAH) is a fatal disease characterized clinically by elevated pulmonary vascular resistance caused extensive proliferation, perivascular inflammation, asymmetric neointimal hyperplasia precapillary arteries. In this review, detailed overview specific role PAH, including its mechanisms activation select ligand, downstream effectors, physiologic effects within tree, provided. Animal models showing importance pathway clinical PAH will be discussed. New drugs biologics that inhibit reverse deadly are highlighted.

Язык: Английский

Процитировано

2

Silencing of NOTCH3 Signaling in Meniscus Smooth Muscle Cells Inhibits Fibrosis and Exacerbates Degeneration in a HEYL‐Dependent Manner DOI Creative Commons
Hao Sun, Fangzhou Liu,

Zhencan Lin

и другие.

Advanced Science, Год журнала: 2023, Номер 10(16)

Опубликована: Апрель 7, 2023

The mechanisms of meniscus fibrosis and novel ways to enhance is unclear. This work reveals human initiated at E24 weeks. Smooth muscle cell cluster identified in embryonic meniscus, the combined analysis with previous data suggests smooth as precursors progenitor cells mature meniscus. NOTCH3 constantly expressed throughout embryogenesis adulthood. Inhibition signaling vivo inhibits exacerbates degeneration. Continuous histological sections show that HEYL, downstream target gene, consistently NOTCH3. HEYL knockdown attenuated COL1A1 upregulation by CTGF TGF-β stimulation. Thus, this study discovers existence fibers a HEYL-dependent manner prevented exacerbated Therefore, NOTCH3/HEYL might be potential therapeutic for fibrosis.

Язык: Английский

Процитировано

4

The relativity analysis of hypoxia inducible factor-1α in pulmonary arterial hypertension (ascites syndrome) in broilers: a review DOI
Weile Fang, Enqi Wang, Pei Liu

и другие.

Avian Pathology, Год журнала: 2024, Номер 53(6), С. 441 - 450

Опубликована: Июнь 18, 2024

Ascites syndrome (AS) in broiler chickens, also known as pulmonary arterial hypertension (PAH), is a significant disease the poultry industry. It nutritional metabolic that closely associated with hypoxia-inducible factors and rapid growth. The rise artery pressure crucial characteristic of AS instrumental its development. Hypoxia-inducible factor 1α (HIF-1α) an active subunit key transcription oxygen-sensing pathway. HIF-1α plays vital role oxygen homeostasis development hypertension. Studying effects on humans or mammals, well ascites broilers, can help us understand pathogenesis AS. Therefore, this review aims to (1) summarize mechanism hypertension, (2) provide theoretical significance explaining (ascites syndrome) (3) establish correlation between broilers. HIGHLIGHTSExplains hypoxic HIF-1α.Linking broilers.Explains microRNAs

Язык: Английский

Процитировано

1

An adult patient with pulmonary arterial hypertension, a NOTCH3 mutation, and leflunomide exposure DOI Creative Commons

Elizabeth G. Fenner,

Catherine E. Simpson

Pulmonary Circulation, Год журнала: 2024, Номер 14(3)

Опубликована: Июль 1, 2024

Abstract Pulmonary arterial hypertension (PAH) is a poorly understood disease of the small pulmonary arteries. vascular remodeling and progressively rising resistance are hallmarks that ultimately result in right heart failure. Several genetic mutations, most notably bone morphogenetic protein receptor type 2, have causal association with heritable forms PAH. Mutations neurogenic locus notch homolog 3 ( NOTCH3) been reported adults children PAH, but whether NOTCH3 causally associated PAH debated. With this case report, we describe clinical characteristics, comorbidities, exposure history an adult patient multiple sclerosis who was found to missense mutation leflunomide.

Язык: Английский

Процитировано

1