RT Journal Article
T1 Role of the Renin-Angiotensin-Aldosterone System in Dystrophin-Deficient Cardiomyopathy
A1 Rodriguez-Gonzalez, Moises
A1 Lubian-Gutierrez, Manuel
A1 Cascales-Poyatos, Helena Maria
A1 Perez-Reviriego, Alvaro Antonio
A1 Castellano-Martinez, Ana
K1 Dystrohinopathy
K1 Duchenne muscular disease
K1 Becker muscular disease
K1 Dystrophic deficient cardiomyopathy
K1 Cardiac fibrosis
K1 Renin angiotensin system
K1 Angiotensin 2
K1 Angiotensin converter enzyme inhibitors
K1 Angiotensin receptor blockers
K1 Enfermedades musculares
K1 Distrofia muscular de Duchenne
K1 Cardiomiopatías
K1 Distrofias musculares
K1 Fibrosis
K1 Sistema renina-angiotensina
K1 Angiotensina II
K1 Inhibidores de la enzima convertidora de angiotensina
K1 Antagonistas de receptores de angiotensina
AB Dystrophin-deficient cardiomyopathy (DDC) is currently the leading cause of death in patients with dystrophinopathies. Targeting myocardial fibrosis (MF) has become a major therapeutic goal in order to prevent the occurrence of DDC. We aimed to review and summarize the current evidence about the role of the renin-angiotensin-aldosterone system (RAAS) in the development and perpetuation of MF in DCC. We conducted a comprehensive search of peer-reviewed English literature on PubMed about this subject. We found increasing preclinical evidence from studies in animal models during the last 20 years pointing out a central role of RAAS in the development of MF in DDC. Local tissue RAAS acts directly mainly through its main fibrotic component angiotensin II (ANG2) and its transducer receptor (AT1R) and downstream TGF-b pathway. Additionally, it modulates the actions of most of the remaining pro-fibrotic factors involved in DDC. Despite limited clinical evidence, RAAS blockade constitutes the most studied, available and promising therapeutic strategy against MF and DDC. Conclusion: Based on the evidence reviewed, it would be recommendable to start RAAS blockade therapy through angiotensin converter enzyme inhibitors (ACEI) or AT1R blockers (ARBs) alone or in combination with mineralocorticoid receptor antagonists (MRa) at the youngest age after the diagnosis of dystrophinopathies, in order to delay the occurrence or slow the progression of MF, even before the detection of any cardiovascular alteration.
PB MDPI
SN 1661-6596
YR 2020
FD 2020-12-31
LK http://hdl.handle.net/10668/4289
UL http://hdl.handle.net/10668/4289
LA en
NO Rodriguez-Gonzalez M, Lubian-Gutierrez M, Cascales-Poyatos HM, Perez-Reviriego AA, Castellano-Martinez A. Role of the Renin-Angiotensin-Aldosterone System in Dystrophin-Deficient Cardiomyopathy. Int J Mol Sci. 2020 Dec 31;22(1):356
DS RISalud
RD Apr 20, 2025