Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
8261605 | Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease | 2012 | 11 Pages |
Abstract
⺠SURF1 mutations are frequent cause of severe COX deficiency. ⺠Altered energy provision leads to increase of respiratory chain complexes I, III, and V. ⺠Adaptive changes in mitochondrial biogenesis are due to posttranscriptional events. ⺠All residual COX is incorporated into I-III2-IV1 supercomplex.
Keywords
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Authors
Nikola KováÅová, Alena ÄÞková Vrbacká, Petr Pecina, Viktor Stránecký, Ewa Pronicka, Stanislav Kmoch, Josef HouÅ¡tÄk,