Nephrotic syndrome (NS), a frequent chronic kidney disease in children and young adults, is the most common phenotype associated with primary coenzyme Q10 (CoQ10) deficiency and is very responsive to CoQ10 supplementation, although the pathomechanism is not clear. Here, using a mouse model of CoQ deficiency-associated NS, we show that long-term oral CoQ10 supplementation prevents kidney failure by rescuing defects of sulfides oxidation and ameliorating oxidative stress, despite only incomplete normalization of kidney CoQ levels and lack of rescue of CoQ-dependent respiratory enzymes activities. Liver and kidney lipidomics, and urine metabolomics analyses, did not show CoQ metabolites. To further demonstrate that sulfides metabolism defects ...
Defects in Coenzyme Q (CoQ) metabolism have been associated with primary mitochondrial disorders, ne...
Therapy of mitochondrial respiratory chain diseases is complicated by limited understanding of cellu...
First demonstration that CoQ10 supplementation is effective for the nephropathy in patients with CoQ...
Coenzyme Q (CoQ) is an electron acceptor for sulfide-quinone reductase (SQR), the first enzyme of th...
Abnormalities of one carbon, glutathione and sulfide metabolisms have recently emerged as novel path...
Coenzyme Q (CoQ) is a key component of the mitochondrial respiratory chain, but it also has several ...
Coenzyme Q (CoQ) is a key component of the mitochondrial respiratory chain, but it also has several ...
Coenzyme Q (CoQ) is a key component of the mitochondrial respiratory chain, but it also has several ...
Primary Coenzyme Q10 (CoQ10) deficiency is an ultra-rare disorder caused by defects in genes involve...
Coenzyme Q (CoQ) is an essential electron carrier in the respiratory chain whose deficiency has been...
none7siPrimary human CoQ10 deficiencies are clinically heterogeneous diseases caused by mutations in...
Coenzyme Q(10) (CoQ(10)) is essential for electron transport in the mitochondrial respiratory chain ...
Coenzyme Q(10) is a remarkable lipid involved in many cellular processes such as energy production t...
Coenzyme Q (CoQ) is an essential component of the mitochondrial electron transport chain and an anti...
Background/Aims Coenzyme Q10 (CoQ10), is a promising antioxidant; however, low bioavailability owing...
Defects in Coenzyme Q (CoQ) metabolism have been associated with primary mitochondrial disorders, ne...
Therapy of mitochondrial respiratory chain diseases is complicated by limited understanding of cellu...
First demonstration that CoQ10 supplementation is effective for the nephropathy in patients with CoQ...
Coenzyme Q (CoQ) is an electron acceptor for sulfide-quinone reductase (SQR), the first enzyme of th...
Abnormalities of one carbon, glutathione and sulfide metabolisms have recently emerged as novel path...
Coenzyme Q (CoQ) is a key component of the mitochondrial respiratory chain, but it also has several ...
Coenzyme Q (CoQ) is a key component of the mitochondrial respiratory chain, but it also has several ...
Coenzyme Q (CoQ) is a key component of the mitochondrial respiratory chain, but it also has several ...
Primary Coenzyme Q10 (CoQ10) deficiency is an ultra-rare disorder caused by defects in genes involve...
Coenzyme Q (CoQ) is an essential electron carrier in the respiratory chain whose deficiency has been...
none7siPrimary human CoQ10 deficiencies are clinically heterogeneous diseases caused by mutations in...
Coenzyme Q(10) (CoQ(10)) is essential for electron transport in the mitochondrial respiratory chain ...
Coenzyme Q(10) is a remarkable lipid involved in many cellular processes such as energy production t...
Coenzyme Q (CoQ) is an essential component of the mitochondrial electron transport chain and an anti...
Background/Aims Coenzyme Q10 (CoQ10), is a promising antioxidant; however, low bioavailability owing...
Defects in Coenzyme Q (CoQ) metabolism have been associated with primary mitochondrial disorders, ne...
Therapy of mitochondrial respiratory chain diseases is complicated by limited understanding of cellu...
First demonstration that CoQ10 supplementation is effective for the nephropathy in patients with CoQ...