Pulmonary vascular dysfunction in end-stage cystic fibrosis: role of NF-κB and endothelin-1

Pulmonary hypertension is rare in chronic respiratory diseases but has a strong impact on the prognosis and is partly underlined by factors other than hypoxaemia. The aim of the present study was to assess the potential role of endothelin-1 (ET-1) and nuclear factor (NF)-κB vasoconstrictive pathways...

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Veröffentlicht in:The European respiratory journal 2009-12, Vol.34 (6), p.1329-1337
Hauptverfasser: HENNO, P, MAUREY, C, DANEL, C, BONNETTE, P, SOUILAMAS, R, STERN, M, DELCLAUX, C, LEVY, M, ISRAËL-BIET, D
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container_end_page 1337
container_issue 6
container_start_page 1329
container_title The European respiratory journal
container_volume 34
creator HENNO, P
MAUREY, C
DANEL, C
BONNETTE, P
SOUILAMAS, R
STERN, M
DELCLAUX, C
LEVY, M
ISRAËL-BIET, D
description Pulmonary hypertension is rare in chronic respiratory diseases but has a strong impact on the prognosis and is partly underlined by factors other than hypoxaemia. The aim of the present study was to assess the potential role of endothelin-1 (ET-1) and nuclear factor (NF)-κB vasoconstrictive pathways in pulmonary hypertension. The effects of ET-1 receptors blockers (BQ 123 and 788) and of genistein were assessed on response to acetylcholine of pulmonary vascular rings from cystic fibrosis (CF) lung transplant recipients (n = 23). NF-κB and ET-1 receptor expression was immunodetected in pulmonary arteries and quantitated using Western blotting. ET-1 vascular content was quantitated using ELISA. In total, 14 out of 23 subjects exhibited strongly impaired pulmonary vasodilation (p
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source Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals
subjects Biological and medical sciences
Chronic obstructive pulmonary disease, asthma
Errors of metabolism
Medical sciences
Metabolic diseases
Miscellaneous hereditary metabolic disorders
Pneumology
title Pulmonary vascular dysfunction in end-stage cystic fibrosis: role of NF-κB and endothelin-1
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