| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 8261626 | Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease | 2012 | 8 Pages |
Abstract
⺠Defective complex I (CI) is the most common type of oxidative phosphorylation disease. ⺠Transcriptomics identified novel molecular processes underlying CI deficiency in fibroblasts of patients. ⺠The Nrf2 pathway is induced in fibroblasts of complex I deficient patients. ⺠Disturbed calcium homeostasis in complex I deficiency can be related to selenoproteins. ⺠Glutathione and selenium metabolism are potentially therapeutic targets in CI deficiency.
Related Topics
Life Sciences
Biochemistry, Genetics and Molecular Biology
Ageing
Authors
A.M. Voets, M. Huigsloot, P.J. Lindsey, A.M. Leenders, W.J.H. Koopman, P.H.G.M. Willems, R.J. Rodenburg, J.A.M. Smeitink, H.J.M. Smeets,