Oxidative stress as a major culprit in kidney disease in diabetes

JM Forbes, MT Coughlan, ME Cooper - Diabetes, 2008 - Am Diabetes Assoc
It is postulated that localized tissue oxidative stress is a key component in the development
of diabetic nephropathy. There remains controversy, however, as to whether this is an early …

Redox control of renal function and hypertension

R Nistala, A Whaley-Connell… - Antioxidants & redox …, 2008 - liebertpub.com
Loss of redox homeostasis and formation of excessive free radicals play an important role in
the pathogenesis of kidney disease and hypertension. Free radicals such as reactive …

CABC1 gene mutations cause ubiquinone deficiency with cerebellar ataxia and seizures

J Mollet, A Delahodde, V Serre, D Chretien… - The American Journal of …, 2008 - cell.com
Coenzyme Q 10 (CoQ 10) plays a pivotal role in oxidative phosphorylation (OXPHOS) in that
it distributes electrons between the various dehydrogenases and the cytochrome segments …

Early coenzyme Q10 supplementation in primary coenzyme Q10 deficiency

G Montini, C Malaventura… - New England Journal of …, 2008 - Mass Medical Soc
To the Editor: Primary coenzyme Q10 deficiency is considered to be the only treatable
mitochondrial disorder, since patients have a response to oral coenzyme Q10 …

[HTML][HTML] Respiratory chain dysfunction and oxidative stress correlate with severity of primary CoQ10 deficiency

CM Quinzii, LC López, J Von-Moltke… - The FASEB journal …, 2008 - ncbi.nlm.nih.gov
Abstract Coenzyme Q 10 (CoQ 10) is essential for electron transport in the mitochondrial
respiratory chain and antioxidant defense. Last year, we reported the first mutations in CoQ …

Current views on collapsing glomerulopathy

M Albaqumi, L Barisoni - Journal of the American Society of …, 2008 - journals.lww.com
Collapsing glomerulopathy is a proliferative disease defined by segmental or global
wrinkling of the glomerular basement membranes associated with podocyte proliferation …

Primary Coenzyme Q Deficiency in Pdss2 Mutant Mice Causes Isolated Renal Disease

M Peng, MJ Falk, VH Haase, R King, E Polyak… - PLoS …, 2008 - journals.plos.org
Coenzyme Q (CoQ) is an essential electron carrier in the respiratory chain whose deficiency
has been implicated in a wide variety of human mitochondrial disease manifestations. Its …

Human CoQ_ {10} deficiencies

CM Quinzii, A Naini, S DiMauro, M Hirano - Biofactors, 2008 - content.iospress.com
Abstract Coenzyme Q [TeX:] _ {10}(CoQ [TeX:] _ {10} or ubiquinone) is a lipid-soluble
component of virtually all cell membranes and has multiple metabolic functions. A major …

Coenzyme Q10 supplementation rescues renal disease in Pdss2kd/kd mice with mutations in prenyl diphosphate synthase subunit 2

R Saiki, AL Lunceford, Y Shi… - American Journal …, 2008 - journals.physiology.org
Homozygous mice carrying kd (kidney disease) mutations in the gene encoding prenyl
diphosphate synthase subunit 2 (Pdss2 kd/kd) develop interstitial nephritis and eventually …

Disorders of mitochondrial function

FG Debray, M Lambert, GA Mitchell - Current opinion in pediatrics, 2008 - journals.lww.com
Mitochondrial diseases can present with signs in almost any organ. Well planned clinical
evaluation is the key to successful diagnostic work-up of mitochondrial diseases. An …