| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 8344043 | Molecular Genetics and Metabolism | 2012 | 9 Pages |
Abstract
⺠Addition of dNMPs resulted in rescue of mtDNA copy numbers in POLG deficient myotubes. ⺠dAMP/dGMP supplementation in DGUOK deficient myotubes improved COX activity. ⺠Substitution of dNMPs may be studied as a therapeutic approach in mtDNA depletion.
Keywords
DGUOKdNDPMNGIEdNMPCOX IIPOLGRNRNRTIPBSdNTPSGMMDSHRPCOXMitochondrial DNAMitochondrial diseaseMitochondrial DNA depletionDeoxyguanosine kinasemtDNARibonucleotide reductasecytochrome c oxidase subunit IIcytochrome c oxidasePhosphate-buffered salinenucleoside reverse transcriptase inhibitorhuman immunodeficiency virusHIVPolymerase gammaPEOProgressive external ophthalmoplegia
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Authors
Stefanie Bulst, Elke Holinski-Feder, Brendan Payne, Angela Abicht, Sabine Krause, Hanns Lochmüller, Patrick F. Chinnery, Maggie C. Walter, Rita Horvath,