Cardiolipin deficiency in Barth syndrome is not associated with increased superoxide/H2O2 production in heart and skeletal muscle mitochondria
| Autor: | Alexander Bartelt, Gökhan S. Hotamisligil, Martin D. Brand, Michael Schlame, Renata L.S. Goncalves |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
tafazzin
Cardiomyopathy Tafazzin Gene Expression Mitochondrion Biochemistry mitochondrial reactive oxygen species Mitochondria Heart chemistry.chemical_compound Mice Structural Biology Superoxides Cardiolipin Mice Knockout 0303 health sciences Gene knockdown biology Superoxide Communication 030302 biochemistry & molecular biology Genetic disorder Barth syndrome mitochondria tazkd mice medicine.medical_specialty ‘ex vivo’ rate of superoxide/H2O2 production Cardiolipins Biophysics Bioenergetics superoxide/H2O2 03 medical and health sciences Oxygen Consumption Internal medicine Genetics medicine Animals Humans Muscle Skeletal Molecular Biology 030304 developmental biology Myocardium Cell Biology Hydrogen Peroxide medicine.disease NAD Mitochondria Muscle Disease Models Animal Endocrinology chemistry Electron Transport Chain Complex Proteins Barth Syndrome biology.protein cardiomyopathy Acyltransferases |
| Zdroj: | Febs Letters |
| ISSN: | 1873-3468 0014-5793 |
| Popis: | Barth syndrome (BTHS) is a rare X-linked genetic disorder caused by mutations in the gene encoding the transacylase tafazzin and characterized by loss of cardiolipin and severe cardiomyopathy. Mitochondrial oxidants have been implicated in the cardiomyopathy in BTHS. Eleven mitochondrial sites produce superoxide/hydrogen peroxide (H2 O2 ) at significant rates. Which of these sites generate oxidants at excessive rates in BTHS is unknown. Here, we measured the maximum capacity of superoxide/H2 O2 production from each site and the ex vivo rate of superoxide/H2 O2 production in the heart and skeletal muscle mitochondria of the tafazzin knockdown mice (tazkd) from 3 to 12 months of age. Despite reduced oxidative capacity, superoxide/H2 O2 production was indistinguishable between tazkd mice and wild-type littermates. These observations raise questions about the involvement of mitochondrial oxidants in BTHS pathology. |
| Databáze: | OpenAIRE |
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