Mitochondrial energy generation disorders: genes, mechanisms, and clues to pathology

AE Frazier, DR Thorburn, AG Compton - Journal of Biological Chemistry, 2019 - ASBMB
Inherited disorders of oxidative phosphorylation cause the clinically and genetically
heterogeneous diseases known as mitochondrial energy generation disorders, or …

The role of mitochondria in insulin resistance and type 2 diabetes mellitus

J Szendroedi, E Phielix, M Roden - Nature Reviews Endocrinology, 2012 - nature.com
Type 2 diabetes mellitus (T2DM) has been related to alterations of oxidative metabolism in
insulin-responsive tissues. Overt T2DM can present with acquired or inherited reductions of …

[HTML][HTML] Choosing between glycolysis and oxidative phosphorylation: a tumor's dilemma?

C Jose, N Bellance, R Rossignol - Biochimica et Biophysica Acta (BBA) …, 2011 - Elsevier
A considerable amount of knowledge has been produced during the last five years on the
bioenergetics of cancer cells, leading to a better understanding of the regulation of energy …

Mitochondrial threshold effects

R Rossignol, B Faustin, C Rocher, M Malgat… - Biochemical …, 2003 - portlandpress.com
The study of mitochondrial diseases has revealed dramatic variability in the phenotypic
presentation of mitochondrial genetic defects. To attempt to understand this variability …

Effect of calcium on the oxidative phosphorylation cascade in skeletal muscle mitochondria

B Glancy, WT Willis, DJ Chess, RS Balaban - Biochemistry, 2013 - ACS Publications
Calcium is believed to regulate mitochondrial oxidative phosphorylation, thereby
contributing to the maintenance of cellular energy homeostasis. Skeletal muscle, with an …

Complex I-mediated reactive oxygen species generation: modulation by cytochrome c and NAD (P)+ oxidation–reduction state

Y Kushnareva, AN Murphy, A Andreyev - Biochemical Journal, 2002 - portlandpress.com
Several lines of evidence indicate that mitochondrial reactive oxygen species (ROS)
generation is the major source of oxidative stress in the cell. It has been shown that ROS …

Oxidative stress, mitochondrial dysfunction and cellular stress response in Friedreich's ataxia

V Calabrese, R Lodi, C Tonon, V D'Agata… - Journal of the …, 2005 - Elsevier
There is significant evidence that the pathogenesis of several neurodegenerative diseases,
including Parkinson's disease, Alzheimer's disease, Friedreich's ataxia (FRDA), multiple …

New insights into doxorubicin-induced cardiotoxicity: the critical role of cellular energetics

M Tokarska-Schlattner, M Zaugg, C Zuppinger… - Journal of molecular and …, 2006 - Elsevier
Cardiotoxic side-effects represent a serious complication of anticancer therapy with
anthracyclines, in particular with doxorubicin (DXR) being the leading drug of the group …

Cytochrome c oxidase and mitochondrial F1F0-ATPase (ATP synthase) activities in platelets and brain from patients with Alzheimer's disease

F Bosetti, F Brizzi, S Barogi, M Mancuso, G Siciliano… - Neurobiology of …, 2002 - Elsevier
Evidence suggests that mitochondrial dysfunction is prominent in Alzheimer's disease (AD).
A failure of one or more of the mitochondrial electron transport chain enzymes or of F1F0 …

Formulation, construction and analysis of kinetic models of metabolism: A review of modelling frameworks

PA Saa, LK Nielsen - Biotechnology advances, 2017 - Elsevier
Kinetic models are critical to predict the dynamic behaviour of metabolic networks.
Mechanistic kinetic models for large networks remain uncommon due to the difficulty of fitting …