Mitochondrial disorders are among the most frequent inborn errors of metabolism, their
primary cause being the dysfunction of the oxidative phosphorylation system (OXPHOS) …

Mitochondria are double‐membrane‐bound organelles that are present in all nucleated
eukaryotic cells and are responsible for the production of cellular energy in the form of ATP …

Complex I (NADH: ubiquinone oxidoreductase) is the largest multimeric enzyme complex of
the mitochondrial respiratory chain, which is responsible for electron transport and the …

Mitochondria contribute to cardiac dysfunction and myocyte injury via a loss of metabolic
capacity and by the production and release of toxic products. This article discusses aspects …

The mitochondrial oxidative phosphorylation (OXPHOS) system is the final biochemical
pathway in the production of ATP. The OXPHOS system consists of five multiprotein …

" This volume inspires. It certainly will be much appreciated by cell biologists all over the
world." Quarterly Review of Biology, March 2009 This book is the eagerly awaited second …

Introduction In the last 10 years the field of mitochondrial genetics has widened, shifting the
focus from rare sporadic, metabolic disease to the effects of mitochondrial DNA (mtDNA) …

Mitochondrial complex I (CI) deficiency is associated with multiple neurological and
metabolic disorders. However, its effect on innate immunity and bone remodeling is unclear …

To study effects of mitochondrial complex I (CI, NADH: ubiquinone oxidoreductase)
deficiency, we inactivated the Ndufs4 gene, which encodes an 18 kDa subunit of the 45 …

Mitochondria are essential for cellular bioenergetics by way of energy production in the form
of ATP through the process of oxidative phosphorylation. This crucial task is executed by five …