Differential responses of pulmonary vascular cells from PAH patients and controls to TNFα and the effect of the BET inhibitor JQ1.


Journal

Respiratory research
ISSN: 1465-993X
Titre abrégé: Respir Res
Pays: England
ID NLM: 101090633

Informations de publication

Date de publication:
29 Jul 2023
Historique:
received: 07 02 2023
accepted: 23 07 2023
medline: 31 7 2023
pubmed: 30 7 2023
entrez: 29 7 2023
Statut: epublish

Résumé

Pulmonary arterial hypertension (PAH) encompasses a group of diseases characterized by raised pulmonary vascular resistance, resulting from vascular remodelling and inflammation. Bromodomain and extra-terminal (BET) proteins are required for the expression of a subset of NF-κB-induced inflammatory genes which can be inhibited by the BET mimic JQ1+. We hypothesised that JQ+ would supress TNFα-driven inflammatory responses in human pulmonary vascular cells from PAH patients. Immunohistochemical staining of human peripheral lung tissue (N = 14 PAH and N = 12 non-PAH) was performed for the BET proteins BRD2 and 4. Human pulmonary microvascular endothelial cells (HPMEC) and pulmonary artery smooth muscle cells (HPASMC) from PAH patients (N = 4) and non-PAH controls (N = 4) were stimulated with TNFα in presence or absence of JQ1+ or its inactive isomer JQ1-. IL-6 and -8 mRNA was measured by RT-qPCR and protein levels by ELISA. Chromatin immunoprecipitation analysis was performed using EZ-ChIP™ and NF-κB p65 activation determined using a TransAm kit. MTT assay was used to measure cell viability. Nuclear staining of BRD2 and BRD4 was significantly (p < 0.0001) increased in the lung vascular endothelial and smooth muscle cells from PAH patients compared to controls with normal lung function. TNFα-driven IL-6 release from both HPMECs and HPASMCs was greater in PAH cells than control cells. Levels of CXCL8/IL-8 protein release was higher in PAH HPASMCs than in control cells with similar release observed in HPMECs. TNFα-induced recruitment of activated NF-κB p65 to the IL-6 and CXCL8/IL-8 promoters were similar in both cell types and between subject groups. JQ1+ suppressed TNFα-induced IL-6 and CXCL8/IL-8 release and mRNA expression to a comparable extent in control and PAH HPMECs and HPASMCs. JQ1 had a greater efficacy on IL-6 release in HPMEC and on CXCL8/IL-8 release in HPASMC. BET inhibition decreases TNFα driven inflammation in primary pulmonary vascular cells. The anti-inflammatory actions of JQ1 suggests distinct cell-specific regulatory control of these genes. BET proteins could be a target for future therapies for PAH.

Sections du résumé

BACKGROUND BACKGROUND
Pulmonary arterial hypertension (PAH) encompasses a group of diseases characterized by raised pulmonary vascular resistance, resulting from vascular remodelling and inflammation. Bromodomain and extra-terminal (BET) proteins are required for the expression of a subset of NF-κB-induced inflammatory genes which can be inhibited by the BET mimic JQ1+. We hypothesised that JQ+ would supress TNFα-driven inflammatory responses in human pulmonary vascular cells from PAH patients.
METHODS METHODS
Immunohistochemical staining of human peripheral lung tissue (N = 14 PAH and N = 12 non-PAH) was performed for the BET proteins BRD2 and 4. Human pulmonary microvascular endothelial cells (HPMEC) and pulmonary artery smooth muscle cells (HPASMC) from PAH patients (N = 4) and non-PAH controls (N = 4) were stimulated with TNFα in presence or absence of JQ1+ or its inactive isomer JQ1-. IL-6 and -8 mRNA was measured by RT-qPCR and protein levels by ELISA. Chromatin immunoprecipitation analysis was performed using EZ-ChIP™ and NF-κB p65 activation determined using a TransAm kit. MTT assay was used to measure cell viability.
RESULTS RESULTS
Nuclear staining of BRD2 and BRD4 was significantly (p < 0.0001) increased in the lung vascular endothelial and smooth muscle cells from PAH patients compared to controls with normal lung function. TNFα-driven IL-6 release from both HPMECs and HPASMCs was greater in PAH cells than control cells. Levels of CXCL8/IL-8 protein release was higher in PAH HPASMCs than in control cells with similar release observed in HPMECs. TNFα-induced recruitment of activated NF-κB p65 to the IL-6 and CXCL8/IL-8 promoters were similar in both cell types and between subject groups. JQ1+ suppressed TNFα-induced IL-6 and CXCL8/IL-8 release and mRNA expression to a comparable extent in control and PAH HPMECs and HPASMCs. JQ1 had a greater efficacy on IL-6 release in HPMEC and on CXCL8/IL-8 release in HPASMC.
CONCLUSION CONCLUSIONS
BET inhibition decreases TNFα driven inflammation in primary pulmonary vascular cells. The anti-inflammatory actions of JQ1 suggests distinct cell-specific regulatory control of these genes. BET proteins could be a target for future therapies for PAH.

Identifiants

pubmed: 37516840
doi: 10.1186/s12931-023-02499-y
pii: 10.1186/s12931-023-02499-y
pmc: PMC10386603
doi:

Substances chimiques

Tumor Necrosis Factor-alpha 0
Interleukin-8 0
(+)-JQ1 compound 0
Interleukin-6 0
NF-kappa B 0
Nuclear Proteins 0
Transcription Factors 0
BRD4 protein, human 0
Cell Cycle Proteins 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

193

Subventions

Organisme : British Heart Foundation
ID : PG/14/27/30679
Pays : United Kingdom
Organisme : British Heart Foundation
ID : PG/14/27/30679
Pays : United Kingdom
Organisme : British Heart Foundation
ID : PG/14/27/30679
Pays : United Kingdom

Informations de copyright

© 2023. The Author(s).

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Auteurs

Sharon Mumby (S)

Respiratory Science, NHLI, Imperial College London, London, UK. s.mumby@imperial.ac.uk.

Frederic Perros (F)

Inserm UMR-S 999, Hôpital Marie Lannelongue, Groupe Hospitalier Paris Saint Joseph, Université Paris-Saclay, Le Plessis-Robinson, France.
CarMeN Laboratory, INSERM U1060, INRAE U1397, Université Claude Bernard Lyon1, Pierre-Bénite, France.

Julien Grynblat (J)

Inserm UMR-S 999, Hôpital Marie Lannelongue, Groupe Hospitalier Paris Saint Joseph, Université Paris-Saclay, Le Plessis-Robinson, France.

Gregoire Manaud (G)

Inserm UMR-S 999, Hôpital Marie Lannelongue, Groupe Hospitalier Paris Saint Joseph, Université Paris-Saclay, Le Plessis-Robinson, France.

Alberto Papi (A)

Interdepartmental Study Center for Inflammatory and Smoke-Related Airway Diseases, Cardiorespiratory and Internal Medicine Section, University of Ferrara, Ferrara, Italy.

Paolo Casolari (P)

Interdepartmental Study Center for Inflammatory and Smoke-Related Airway Diseases, Cardiorespiratory and Internal Medicine Section, University of Ferrara, Ferrara, Italy.

Gaetano Caramori (G)

Pneumologia, Dipartimento di Scienze Biomediche, Odontoiatriche e Delle Immagini Morfologiche e Funzionali (BIOMORF), Università Degli Studi di Messina, Messina, Italy.

Marc Humbert (M)

Inserm UMR-S 999, Hôpital Marie Lannelongue, Groupe Hospitalier Paris Saint Joseph, Université Paris-Saclay, Le Plessis-Robinson, France.
Department of Respiratory and Intensive Care Medicine, AP-HP, Hôpital Bicêtre, Pulmonary Hypertension National Referral Center, Université Paris-Saclay, Le Kremlin-Bicêtre, France.

S John Wort (S)

Respiratory Science, NHLI, Imperial College London, London, UK.
National Pulmonary Hypertension Service, Royal Brompton Hospital, London, UK.

Ian M Adcock (IM)

Respiratory Science, NHLI, Imperial College London, London, UK.

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