Early impairment in mitochondrial quality check and function precedes the development of cardiac phenotypes in an mouse model of Fabry Disease

Jessica Gambardella; Antonella Fiordelisi; Federica Andrea Cerasuolo; Antonietta Buonaiuto; Roberta Avvisato; Alessandro Viti; Eduardo Sommella; Pietro Campiglia; Valeria D'Argenio; Nella Prevete; Stefania D'Apice; Fahimeh Varzideh; Roberta Paolillo; Cinzia Perrino; Eleonora Riccio; Antonio Pisani; Antonio Bianco; Junichi Sadoshima; Letizia Spinelli; Gaetano Santulli; Daniela Sorriento; Guido Iaccarino

doi:10.1016/j.vph.2024.107320

Early impairment in mitochondrial quality check and function precedes the development of cardiac phenotypes in an mouse model of Fabry Disease

Vascul Pharmacol. 2024 Jun:155:107320. doi: 10.1016/j.vph.2024.107320.

Authors

Jessica Gambardella¹, Antonella Fiordelisi², Federica Andrea Cerasuolo², Antonietta Buonaiuto², Roberta Avvisato², Alessandro Viti², Eduardo Sommella³, Pietro Campiglia³, Valeria D'Argenio⁴, Nella Prevete⁵, Stefania D'Apice², Fahimeh Varzideh⁶, Roberta Paolillo², Cinzia Perrino², Eleonora Riccio⁷, Antonio Pisani⁷, Antonio Bianco⁸, Junichi Sadoshima⁹, Letizia Spinelli⁸, Gaetano Santulli¹⁰, Daniela Sorriento¹, Guido Iaccarino¹

Affiliations

¹ Department of Advanced Biomedical Sciences, Federico II University, 80131 Naples, Italy; Interdepartmental Center of Research on Hypertension and Related Conditions (CIRIAPA), Federico II University, 80131 Naples, Italy.
² Department of Advanced Biomedical Sciences, Federico II University, 80131 Naples, Italy.
³ Department of Pharmacy, University of Salerno, 84084 Salerno, Italy.
⁴ Department of Human Sciences and Quality of Life Promotion, San Raffaele Open University, 00166 Rome, Italy; CEINGE- Advanced Biotechnologies, 80131 Naples, Italy.
⁵ Department of Molecular Medicine and Medical Biotechnologies, Federico II University, 80131 Naples, Italy.
⁶ Department of Molecular Pharmacology, Fleischer Institute for Diabetes and Metabolism (FIDAM), Einstein-Mount Sinai Diabetes Research Center (ES-DRC), Einstein Institute for Neuroimmunology and Inflammation, Albert Einstein College of Medicine, 10461 New York, NY, USA.
⁷ Department of Public Health, Federico II University, 80131 Naples, Italy.
⁸ Interdepartmental Center of Research on Hypertension and Related Conditions (CIRIAPA), Federico II University, 80131 Naples, Italy.
⁹ Department of Cell Biology and Molecular Medicine, New Jersey Medical School, Rutgers University, 07103, NJ, USA.
¹⁰ Department of Advanced Biomedical Sciences, Federico II University, 80131 Naples, Italy; Interdepartmental Center of Research on Hypertension and Related Conditions (CIRIAPA), Federico II University, 80131 Naples, Italy; Department of Molecular Pharmacology, Fleischer Institute for Diabetes and Metabolism (FIDAM), Einstein-Mount Sinai Diabetes Research Center (ES-DRC), Einstein Institute for Neuroimmunology and Inflammation, Albert Einstein College of Medicine, 10461 New York, NY, USA; Department of Medicine, Division of Cardiology, Wilf Family Cardiovascular Research Institute, Einstein Institute for Aging Research, Albert Einstein College of Medicine, 10461 New York, NY, USA.

PMID: 38985588
DOI: 10.1016/j.vph.2024.107320

No abstract available

MeSH terms

Animals
Disease Models, Animal*
Fabry Disease* / genetics
Fabry Disease* / metabolism
Fabry Disease* / pathology
Fabry Disease* / physiopathology
Male
Mice
Mice, Inbred C57BL
Mitochondria, Heart* / metabolism
Mitochondria, Heart* / pathology
Myocytes, Cardiac / metabolism
Myocytes, Cardiac / pathology
Phenotype*
alpha-Galactosidase / genetics
alpha-Galactosidase / metabolism

Substances

alpha-Galactosidase