Journal
MITOCHONDRION
Volume 12, Issue 4, Pages 465-471Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.mito.2012.06.006
Keywords
Mitochondrial DNA; T1095C; 12SrRNA; Aminoglycosides; Apoptosis; Mitochondrial parkinsonism
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Funding
- National Health and Medical Research Council (Australia)
- Flinders Medical Centre Foundation
- Flinders University
- Grants-in-Aid for Scientific Research [22240072] Funding Source: KAKEN
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We have previously reported a heteroplasmic mtDNA mutation (T1095C) in the 12SrRNA gene of an Italian family with features of maternally-inherited parkinsonism, antibiotic-mediated deafness and peripheral neuropathy. In the present study, we demonstrate that a transmitochondrial cybrid line derived from the proband of this family shows selective depletion of mitochondrial glutathione and decreases in the activity of complex Moreover, when exposed to an aminoglycoside antibiotic these cells responded with a ten-fold increase in the number of apoptotic cells compared to controls. These results support a pathogenic role for the T1095C mutation and indicate that the mutation increases the risk for aminoglycoside-induced toxicity. (C) 2012 Elsevier B.V. and Mitochondria Research Society. All rights reserved.
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