Hypertension and Vasculopathies

Popper, Helmut; Murer, Bruno

doi:10.1007/978-3-030-22664-0_34

Cited by 1 publication

(2 citation statements)

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“…The Fulton index minimally changed over the course of hypoxia in Notch3 −/− mice but significantly increased in Notch3 +/+ mice [ 8 ]. After exposure to chronic hypoxia, Notch3 +/+ mice developed excessive small pulmonary artery muscularization and luminal narrowing, consistent with advanced pulmonary hypertension [ 40 ], whereas Notch3 −/− mice had normal-appearing small arteries without muscular thickening [ 8 ]. Although measurable pulmonary arterial wall thickness in small pulmonary arteries increased in Notch3 +/+ mice compared to Notch3 −/− mice, there was no significant difference between the groups in the vessel/alveoli ratios [ 8 ].…”

Section: Notch3 −/− Mice Do Not Develop Phmentioning

confidence: 99%

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NOTCH3 and Pulmonary Arterial Hypertension

Winicki,

Puerta,

Besse

et al. 2024

IJMS

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NOTCH3 receptor signaling has been linked to the regulation of smooth muscle cell proliferation and the maintenance of smooth muscle 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 by extensive vascular smooth muscle cell proliferation, perivascular inflammation, and asymmetric neointimal hyperplasia in precapillary pulmonary arteries. In this review, a detailed overview of the specific role of NOTCH3 signaling in PAH, including its mechanisms of activation by a select ligand, downstream signaling effectors, and physiologic effects within the pulmonary vascular tree, is provided. Animal models showing the importance of the NOTCH3 pathway in clinical PAH will be discussed. New drugs and biologics that inhibit NOTCH3 signaling and reverse this deadly disease are highlighted.

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Section: Notch3 −/− Mice Do Not Develop Phmentioning

confidence: 99%

“…Furthermore, Notch3 −/− mice exhibited normal pulmonary angiograms with diffuse vascular blush [ 8 ]. Conversely, Notch3 +/+ littermates displayed angiograms indicative of severe small-vessel pruning [ 8 ] similar to that seen in human PAH [ 40 ].…”

Section: Notch3 −/− Mice Do Not Develop Phmentioning

confidence: 99%