Mechanisms Underlying Development of Taurine-Deficient Cardiomyopathy
| Autor: | Chian Ju Jong, Jay H. Kramer, Takashi Ito, Stephen W. Schaffer, Junichi Azuma |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
0301 basic medicine
medicine.medical_specialty Taurine 030102 biochemistry & molecular biology business.industry Mitochondrial disease Cardiomyopathy Respiratory chain Mitochondrion medicine.disease 03 medical and health sciences chemistry.chemical_compound 030104 developmental biology Endocrinology Mitochondrial permeability transition pore chemistry Internal medicine Lactic acidosis Heart failure medicine business |
| Zdroj: | Hearts. 1:86-98 |
| ISSN: | 2673-3846 |
| DOI: | 10.3390/hearts1020010 |
| Popis: | Taurine is a ubiquitous β-amino acid that plays an essential role in ensuring normal mitochondrial and myocardial function. In the mitochondria, taurine reacts with a tRNA forming a 5-taurinomethyluridine conjugate that primarily regulates the biosynthesis of the mitochondria encoded protein, ND6, which serves as a subunit of complex I of the respiratory chain. Impaired formation of the taurine conjugate reduces activity of complex I and plays a central role in the pathophysiology of the mitochondrial disease MELAS (myopathy, encephalopathy, lactic acidosis and stroke-like episodes). The restoration of mitochondrial levels of the taurine conjugate enhances electron flux through the respiratory chain, thereby preventing at least some of the symptoms of MELAS. Taurine therapy also diminishes the severity of congestive heart failure, an observation that led to its approval for the treatment of congestive heart failure in Japan. The review article discusses the role of defective calcium handling, reduced ATP generation, enhanced oxidative stress and apoptosis in the development of taurine-deficient cardiomyopathy. Some patients suffering from congestive heart failure are taurine-deficient, an observation supporting the hypothesis that low taurine levels contribute to the severity of heart failure. Thus, mishandling of taurine leads to mitochondrial dysfunction, which is involved in the development of both MELAS and congestive heart failure. |
| Databáze: | OpenAIRE |
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