Protective effects of rimeporide on left ventricular function in golden retriever muscular dystrophy dogs.

Canine model of Duchenne muscular dystrophy Echocardiography Left ventricular function Na(+)-H(+) exchanger isoform 1 Rimeporide

Journal

International journal of cardiology
ISSN: 1874-1754
Titre abrégé: Int J Cardiol
Pays: Netherlands
ID NLM: 8200291

Informations de publication

Date de publication:
01 08 2020
Historique:
received: 19 11 2019
revised: 24 02 2020
accepted: 11 03 2020
pubmed: 23 3 2020
medline: 15 5 2021
entrez: 23 3 2020
Statut: ppublish

Résumé

Alterations in intracellular Na Golden retriever muscular dystrophy (GRMD) dogs were randomized to orally receive rimeporide (10 mg/kg, twice a day) or placebo from 2 months to 1 year of age. Left ventricular (LV) function was assessed by conventional and advanced echocardiography. Compared with placebo-treated GRMD, LV function deterioration with age was limited in rimeporide-treated GRMD dogs as indicated by the preservation of LV ejection fraction as well as overall cardiac parameters different from placebo-treated dogs, as revealed by composite cardiac scores and principal component analysis. In addition, principal component analysis clustered rimeporide-treated GRMD dogs close to healthy control dogs. Chronic administration of the NHE1 inhibitor rimeporide exerted a protective effect against LV function decline in GRMD dogs. This study provides proof of concept to explore the cardiac effects of rimeporide in DMD patients.

Sections du résumé

BACKGROUND
Alterations in intracellular Na
METHODS
Golden retriever muscular dystrophy (GRMD) dogs were randomized to orally receive rimeporide (10 mg/kg, twice a day) or placebo from 2 months to 1 year of age. Left ventricular (LV) function was assessed by conventional and advanced echocardiography.
RESULTS
Compared with placebo-treated GRMD, LV function deterioration with age was limited in rimeporide-treated GRMD dogs as indicated by the preservation of LV ejection fraction as well as overall cardiac parameters different from placebo-treated dogs, as revealed by composite cardiac scores and principal component analysis. In addition, principal component analysis clustered rimeporide-treated GRMD dogs close to healthy control dogs.
CONCLUSIONS
Chronic administration of the NHE1 inhibitor rimeporide exerted a protective effect against LV function decline in GRMD dogs. This study provides proof of concept to explore the cardiac effects of rimeporide in DMD patients.

Identifiants

pubmed: 32199683
pii: S0167-5273(19)35776-6
doi: 10.1016/j.ijcard.2020.03.031
pii:
doi:

Substances chimiques

Anti-Arrhythmia Agents 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

89-95

Commentaires et corrections

Type : CommentIn

Informations de copyright

Copyright © 2020 Elsevier B.V. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of competing interest F. Porte Thomé and T. D. Tran are employees of the EspeRare, a not-for-profit foundation. F. Porte Thomé is a co-founder of the EspeRare foundation. Employees and founders of the EspeRare Foundation do not own stock or options. The patent on rimeporide has expired and these results will not lead to patent prosecution. The other authors have nothing to declare.

Auteurs

Bijan Ghaleh (B)

U955-IMRB, Equipe 03, Inserm, UPEC, Ecole Nationale Vétérinaire d'Alfort, Maisons-Alfort, France.

Inès Barthélemy (I)

U955-IMRB, Equipe 10, Inserm, UPEC, Ecole Nationale Vétérinaire d'Alfort, Maisons-Alfort, France.

Jérôme Wojcik (J)

Translational Informatics & Biometrics Europe, Precision for Medicine, CH-1202 Geneva, Switzerland.

Lucien Sambin (L)

U955-IMRB, Equipe 03, Inserm, UPEC, Ecole Nationale Vétérinaire d'Alfort, Maisons-Alfort, France.

Alain Bizé (A)

U955-IMRB, Equipe 03, Inserm, UPEC, Ecole Nationale Vétérinaire d'Alfort, Maisons-Alfort, France.

Luc Hittinger (L)

U955-IMRB, Equipe 10, Inserm, UPEC, Ecole Nationale Vétérinaire d'Alfort, Maisons-Alfort, France; AP-HP, Hôpitaux Universitaires Henri Mondor, Fédération de Cardiologie, F-94000 Créteil, France.

Thien Duc Tran (TD)

EspeRare, Campus Biotech Innovation Park, Avenue de Secheron 15, 1202 Geneva, Switzerland.

Florence Porte Thomé (FP)

EspeRare, Campus Biotech Innovation Park, Avenue de Secheron 15, 1202 Geneva, Switzerland.

Stéphane Blot (S)

U955-IMRB, Equipe 10, Inserm, UPEC, Ecole Nationale Vétérinaire d'Alfort, Maisons-Alfort, France.

Jin Bo Su (JB)

U955-IMRB, Equipe 03, Inserm, UPEC, Ecole Nationale Vétérinaire d'Alfort, Maisons-Alfort, France. Electronic address: jin-bo.su@inserm.fr.

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Classifications MeSH