Fabry Disease: Cardiac Implications and Molecular Mechanisms.
Curr Heart Fail Rep
; 21(2): 81-100, 2024 04.
Article
en En
| MEDLINE
| ID: mdl-38289538
ABSTRACT
PURPOSE OF REVIEW This review explores the interplay among metabolic dysfunction, oxidative stress, inflammation, and fibrosis in Fabry disease, focusing on their potential implications for cardiac involvement. We aim to discuss the biochemical processes that operate in parallel to sphingolipid accumulation and contribute to disease pathogenesis, emphasizing the importance of a comprehensive understanding of these processes. RECENT FINDINGS:
Beyond sphingolipid accumulation, emerging studies have revealed that mitochondrial dysfunction, oxidative stress, and chronic inflammation could be significant contributors to Fabry disease and cardiac involvement. These factors promote cardiac remodeling and fibrosis and may predispose Fabry patients to conduction disturbances, ventricular arrhythmias, and heart failure. While current treatments, such as enzyme replacement therapy and pharmacological chaperones, address disease progression and symptoms, their effectiveness is limited. Our review uncovers the potential relationships among metabolic disturbances, oxidative stress, inflammation, and fibrosis in Fabry disease-related cardiac complications. Current findings suggest that beyond sphingolipid accumulation, other mechanisms may significantly contribute to disease pathogenesis. This prompts the exploration of innovative therapeutic strategies and underscores the importance of a holistic approach to understanding and managing Fabry disease.Palabras clave
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Enfermedad de Fabry
/
Insuficiencia Cardíaca
Límite:
Humans
Idioma:
En
Revista:
Curr Heart Fail Rep
Asunto de la revista:
CARDIOLOGIA
Año:
2024
Tipo del documento:
Article
País de afiliación:
Alemania