Effect of Coenzyme Q10 supplementation on mitochondrial electron transport chain activity and mitochondrial oxidative stress in Coenzyme Q10 deficient human neuronal cells.

[1]  S. Rahman,et al.  Human neuronal coenzyme Q10 deficiency results in global loss of mitochondrial respiratory chain activity, increased mitochondrial oxidative stress and reversal of ATP synthase activity: implications for pathogenesis and treatment , 2013, Journal of Inherited Metabolic Disease.

[2]  S. Dimauro,et al.  Heterogeneity of coenzyme Q10 deficiency: patient study and literature review. , 2012, Archives of neurology.

[3]  S. Rahman,et al.  176th ENMC International Workshop: Diagnosis and treatment of coenzyme Q10 deficiency , 2012, Neuromuscular Disorders.

[4]  D. Turnbull,et al.  Mechanism of neurodegeneration of neurons with mitochondrial DNA mutations , 2010, Brain : a journal of neurology.

[5]  P. Navas,et al.  Analysis of coenzyme Q10 in muscle and fibroblasts for the diagnosis of CoQ10 deficiency syndromes. , 2008, Clinical biochemistry.

[6]  M. V. Miles.  The uptake and distribution of coenzyme Q(10) , 2007 .

[7]  K. Brismar,et al.  The antioxidant role of coenzyme Q. , 2007, Mitochondrion.

[8]  S. Dimauro,et al.  Missense mutation of the COQ2 gene causes defects of bioenergetics and de novo pyrimidine synthesis. , 2007, Human molecular genetics.

[9]  R. Chopra,et al.  Coenzyme Q10: Absorption, tissue uptake, metabolism and pharmacokinetics , 2006, Free radical research.

[10]  K. Mills,et al.  Determination of coenzyme Q10 status in blood mononuclear cells, skeletal muscle, and plasma by HPLC with di-propoxy-coenzyme Q10 as an internal standard. , 2005, Clinical chemistry.

[11]  T. Chojnacki,et al.  Distribution and breakdown of labeled coenzyme Q10 in rat. , 2003, Free radical biology & medicine.

[12]  B. Fanburg,et al.  Reactive oxygen species in cell signaling. , 2000, American journal of physiology. Lung cellular and molecular physiology.

[13]  M. Selak,et al.  Mitochondrial activity in Pompe's disease. , 2000, Pediatric neurology.

[14]  A. Vercesi,et al.  Mitochondrial damage induced by conditions of oxidative stress. , 1999, Free radical biology & medicine.

[15]  L S Freedman,et al.  Multiple neurotransmitter synthesis by human neuroblastoma cell lines and clones. , 1978, Cancer research.

[16]  H. Rudney,et al.  J-Hydroxybenzoate: polyprenyl transferase and the prenylation of 4-aminobenzoate in mammalian tissues. , 1975, Archives of biochemistry and biophysics.

[17]  Oliver H. Lowry,et al.  Protein measurement with the Folin phenol reagent. , 1951, The Journal of biological chemistry.

[18]  M. V. Miles.  The uptake and distribution of coenzyme Q10. , 2007, Mitochondrion.