| First Author | Paterson AW | Year | 2008 |
| Journal | J Neurochem | Volume | 105 |
| Issue | 1 | Pages | 177-91 |
| PubMed ID | 17999717 | Mgi Jnum | J:134896 |
| Mgi Id | MGI:3790022 | Doi | 10.1111/j.1471-4159.2007.05123.x |
| Citation | Paterson AW, et al. (2008) Complex I specific increase in superoxide formation and respiration rate by PrP-null mouse brain mitochondria. J Neurochem 105(1):177-91 |
| abstractText | An imbalance in free radical production and removal is considered by many to be an important factor in the etiology of many degenerative diseases. Since mitochondria are a major source of free radicals, we have examined mitochondrial free radical production in relation to oxidative phosphorylation in PrP-null mice. Quantitative electron paramagnetic resonance spectroscopy revealed up to a 70% increase in superoxide production from Complex I of submitochondrial particles prepared from PrP-null mice. This was accompanied by elevated respiratory capacity through Complex I without any discernible alteration in respiratory efficiency. These differences are associated with changes in superoxide dismutase levels and defects in mitochondrial morphology, confirming previously reported results. Our results demonstrate a clear difference in free radical production and oxygen consumption by mitochondrial Complex I between PrP-null mice and wild-type controls, pointing to Complex I as a potential target for pathological change, suggesting similarities between prion-related and other neurodegenerative diseases. |
