Heterogeneity of coenzyme Q10 deficiency: patient study and literature review
Coenzyme Q 10 (CoQ 10) deficiency has been associated with 5 major clinical phenotypes:
encephalomyopathy, severe infantile multisystemic disease, nephropathy, cerebellar ataxia …
encephalomyopathy, severe infantile multisystemic disease, nephropathy, cerebellar ataxia …
Renal involvement in mitochondrial cytopathies
Mitochondrial cytopathies constitute a group of rare diseases that are characterized by their
frequent multisystemic involvement, extreme variability of phenotype and complex genetics …
frequent multisystemic involvement, extreme variability of phenotype and complex genetics …
Ubiquinone (coenzyme Q10) prevents renal mitochondrial dysfunction in an experimental model of type 2 diabetes
KC Sourris, BE Harcourt, PH Tang, AL Morley… - Free Radical Biology …, 2012 - Elsevier
Cardiovascular benefits of ubiquinone have been previously demonstrated, and we
administered it as a novel therapy in an experimental model of type 2 diabetic nephropathy …
administered it as a novel therapy in an experimental model of type 2 diabetic nephropathy …
[HTML][HTML] How many ways can a podocyte die?
PL Tharaux, TB Huber - Seminars in nephrology, 2012 - Elsevier
Podocytes are highly specialized epithelial cells that line the urinary surface of the
glomerular capillary tuft. To maintain kidney filtration, podocytes oppose the high …
glomerular capillary tuft. To maintain kidney filtration, podocytes oppose the high …
176th ENMC International Workshop: diagnosis and treatment of coenzyme Q10 deficiency
An ENMC meeting was held in Naarden, the Netherlands (July 9th–11th 2010) with 12
clinical and basic scientists and three industrial representatives from Europe (France …
clinical and basic scientists and three industrial representatives from Europe (France …
CoQ10 deficiencies and MNGIE: two treatable mitochondrial disorders
BACKGROUND: Although causative mutations have been identified for numerous
mitochondrial disorders, few disease-modifying treatments are available. Two examples of …
mitochondrial disorders, few disease-modifying treatments are available. Two examples of …
Pathobiology of focal segmental glomerulosclerosis: new developments
VD D'Agati - Current opinion in nephrology and hypertension, 2012 - journals.lww.com
FSGS is caused by a complex interplay of inherent genetic susceptibilities and external
injurious factors acting on podocytes. Critical levels of podocyte stress eventuate in …
injurious factors acting on podocytes. Critical levels of podocyte stress eventuate in …
Progress in pathogenesis of proteinuria
A Zhang, S Huang - International journal of nephrology, 2012 - Wiley Online Library
Aims. Proteinuria not only is a sign of kidney damage, but also is involved in the progression
of renal diseases as an independent pathologic factor. Clinically, glomerular proteinuria is …
of renal diseases as an independent pathologic factor. Clinically, glomerular proteinuria is …
Update on clinical aspects and treatment of selected vitamin-responsive disorders II (riboflavin and CoQ10)
R Horvath - Journal of inherited metabolic disease, 2012 - Springer
Riboflavin and ubiquinone (Coenzyme Q 10, CoQ 10) deficiencies are heterogeneous
groups of autosomal recessive conditions affecting both children and adults. Riboflavin …
groups of autosomal recessive conditions affecting both children and adults. Riboflavin …
Effects of Inhibiting CoQ10 Biosynthesis with 4-nitrobenzoate in Human Fibroblasts
CM Quinzii, S Tadesse, A Naini, M Hirano - PloS one, 2012 - journals.plos.org
Coenzyme Q10 (CoQ10) is a potent lipophilic antioxidant in cell membranes and a carrier of
electrons in the mitochondrial respiratory chain. We previously characterized the effects of …
electrons in the mitochondrial respiratory chain. We previously characterized the effects of …