[HTML][HTML] Mitochondrial management of reactive oxygen species

G Napolitano, G Fasciolo, P Venditti - Antioxidants, 2021 - mdpi.com
Mitochondria in aerobic eukaryotic cells are both the site of energy production and the
formation of harmful species, such as radicals and other reactive oxygen species, known as …

[HTML][HTML] Patient care standards for primary mitochondrial disease: a consensus statement from the Mitochondrial Medicine Society

S Parikh, A Goldstein, A Karaa, MK Koenig, I Anselm… - Genetics in …, 2017 - Elsevier
The purpose of this statement is to provide consensus-based recommendations for optimal
management and care for patients with primary mitochondrial disease. This statement is …

[HTML][HTML] Treatable inborn errors of metabolism causing intellectual disability: a systematic literature review

CDM van Karnebeek, S Stockler - Molecular genetics and metabolism, 2012 - Elsevier
BACKGROUND: Intellectual disability ('developmental delay'at age< 5years) affects 2.5% of
population worldwide. Recommendations to investigate genetic causes of intellectual …

Mechanisms of mitochondrial diseases

E Ylikallio, A Suomalainen - Annals of medicine, 2012 - Taylor & Francis
Mitochondria are essential organelles with multiple functions, the most well known being the
production of adenosine triphosphate (ATP) through oxidative phosphorylation (OXPHOS) …

[HTML][HTML] Metabolic regulation to treat bipolar depression: mechanisms and targeting by trimetazidine

S Khanra, P Reddy, A Giménez-Palomo… - Molecular …, 2023 - nature.com
Bipolar disorder's core feature is the pathological disturbances in mood, often accompanied
by disrupted thinking and behavior. Its complex and heterogeneous etiology implies that a …

Primary and secondary coenzyme Q10 deficiency: the role of therapeutic supplementation

M Potgieter, E Pretorius, MS Pepper - Nutrition reviews, 2013 - academic.oup.com
Abstract Coenzyme Q10 (CoQ10) is the only lipid-soluble antioxidant that animal cells
synthesize de novo. It is found in cell membranes and is particularly well known for its role in …

Mitochondrial protein functions elucidated by multi-omic mass spectrometry profiling

JA Stefely, NW Kwiecien, EC Freiberger… - Nature …, 2016 - nature.com
Mitochondrial dysfunction is associated with many human diseases, including cancer and
neurodegeneration, that are often linked to proteins and pathways that are not well …

Mitochondrial COQ9 is a lipid-binding protein that associates with COQ7 to enable coenzyme Q biosynthesis

DC Lohman, F Forouhar, ET Beebe… - Proceedings of the …, 2014 - National Acad Sciences
Coenzyme Q (CoQ) is an isoprenylated quinone that is essential for cellular respiration and
is synthesized in mitochondria by the combined action of at least nine proteins (COQ1–9) …

[PDF][PDF] Cerebellar ataxia and coenzyme Q deficiency through loss of unorthodox kinase activity

JA Stefely, F Licitra, L Laredj, AG Reidenbach… - Molecular cell, 2016 - cell.com
The UbiB protein kinase-like (PKL) family is widespread, comprising one-quarter of
microbial PKLs and five human homologs, yet its biochemical activities remain obscure …

Droplet size and composition of nutraceutical nanoemulsions influences bioavailability of long chain fatty acids and Coenzyme Q10

HT Cho, L Salvia-Trujillo, J Kim, Y Park, H Xiao… - Food Chemistry, 2014 - Elsevier
The influence of droplet size (d 32= 0.21, 0.70 or 2.2 μm) and oil digestibility (corn oil versus
mineral oil) on the bioavailability of a model long chain fatty acid (heptadecanoic acid) and …