Neuroprotective effect of antioxidants in the brain

KH Lee, M Cha, BH Lee - International journal of molecular sciences, 2020 - mdpi.com
The brain is vulnerable to excessive oxidative insults because of its abundant lipid content,
high energy requirements, and weak antioxidant capacity. Reactive oxygen species (ROS) …

Mitochondrial disorders of the OXPHOS system

E Fernandez‐Vizarra, M Zeviani - FEBS letters, 2021 - Wiley Online Library
Mitochondrial disorders are among the most frequent inborn errors of metabolism, their
primary cause being the dysfunction of the oxidative phosphorylation system (OXPHOS) …

Mitochondrial dysfunction in inherited renal disease and acute kidney injury

F Emma, G Montini, SM Parikh, L Salviati - Nature Reviews Nephrology, 2016 - nature.com
Mitochondria are increasingly recognized as key players in genetic and acquired renal
diseases. Most mitochondrial cytopathies that cause renal symptoms are characterized by …

Exploring the genetic basis of early-onset chronic kidney disease

A Vivante, F Hildebrandt - Nature Reviews Nephrology, 2016 - nature.com
The primary causes of chronic kidney disease (CKD) in children differ from those of CKD in
adults. In the USA the most common diagnostic groups of renal disease that manifest before …

Mitochondrial dysfunction in the pathophysiology of renal diseases

R Che, Y Yuan, S Huang… - American Journal of …, 2014 - journals.physiology.org
Mitochondrial dysfunction has gained recognition as a contributing factor in many diseases.
The kidney is a kind of organ with high energy demand, rich in mitochondria. As such …

[HTML][HTML] Coenzyme Q biosynthesis in health and disease

MJ Acosta, LV Fonseca, MA Desbats, C Cerqua… - … et Biophysica Acta (BBA …, 2016 - Elsevier
Coenzyme Q (CoQ, or ubiquinone) is a remarkable lipid that plays an essential role in
mitochondria as an electron shuttle between complexes I and II of the respiratory chain, and …

ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption

S Ashraf, HY Gee, S Woerner, LX Xie… - The Journal of …, 2013 - Am Soc Clin Investig
Identification of single-gene causes of steroid-resistant nephrotic syndrome (SRNS) has
furthered the understanding of the pathogenesis of this disease. Here, using a combination …

COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness

SF Heeringa, G Chernin, M Chaki… - The Journal of …, 2011 - Am Soc Clin Investig
Steroid-resistant nephrotic syndrome (SRNS) is a frequent cause of end-stage renal failure.
Identification of single-gene causes of SRNS has generated some insights into its …

Genetic bases and clinical manifestations of coenzyme Q10 (CoQ10) deficiency

MA Desbats, G Lunardi, M Doimo, E Trevisson… - Journal of inherited …, 2015 - Springer
Coenzyme Q 10 is a remarkable lipid involved in many cellular processes such as energy
production through the mitochondrial respiratory chain (RC), beta-oxidation of fatty acids …

Clinical aspects of coenzyme Q10: an update

GP Littarru, L Tiano - Nutrition, 2010 - Elsevier
The fundamental role of coenzyme Q10 (CoQ10) in mitochondrial bioenergetics and its well-
acknowledged antioxidant properties constitute the basis for its clinical applications …