Coenzyme Q10 supplementation rescues renal disease in Pdss2kd/kd mice with mutations in prenyl diphosphate synthase subunit 2 - Test2 - elhamkhani - TriplyDB

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

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

http://www.wikidata.org/entity/Q36977423

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COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness.Genotype-phenotype correlations in non-Finnish congenital nephrotic syndrome A conserved START domain coenzyme Q-binding polypeptide is required for efficient Q biosynthesis, respiratory electron transport, and antioxidant function in Saccharomyces cerevisiae.Expression of the human atypical kinase ADCK3 rescues coenzyme Q biosynthesis and phosphorylation of Coq polypeptides in yeast coq8 mutants Treatment of CoQ(10) deficient fibroblasts with ubiquinone, CoQ analogs, and vitamin C: time- and compound-dependent effects A cis-regulatory mutation of PDSS2 causes silky-feather in chickens Molecular characterization of the human COQ5 C-methyltransferase in coenzyme Q10 biosynthesis.Mitochondrial dysfunction in inherited renal disease and acute kidney injury Effects of inhibiting CoQ10 biosynthesis with 4-nitrobenzoate in human fibroblasts.Reactive oxygen species, oxidative stress, and cell death correlate with level of CoQ10 deficiency.The podocyte power-plant disaster and its contribution to glomerulopathy.Renal mitochondrial cytopathies.Mitochondrial Diseases Part I: mouse models of OXPHOS deficiencies caused by defects in respiratory complex subunits or assembly factors.176th ENMC International Workshop: diagnosis and treatment of coenzyme Q₁₀ deficiency Parkinson's disease-like neuromuscular defects occur in prenyl diphosphate synthase subunit 2 (Pdss2) mutant mice.CoQ(10) deficiencies and MNGIE: two treatable mitochondrial disorders.Progress in pathogenesis of proteinuria.Coenzyme Q10 restores oocyte mitochondrial function and fertility during reproductive aging Probucol ameliorates renal and metabolic sequelae of primary CoQ deficiency in Pdss2 mutant mice Restoring de novo coenzyme Q biosynthesis in Caenorhabditis elegans coq-3 mutants yields profound rescue compared to exogenous coenzyme Q supplementation.Tissue-specific oxidative stress and loss of mitochondria in CoQ-deficient Pdss2 mutant mice.Focal segmental glomerulosclerosis is associated with a PDSS2 haplotype and, independently, with a decreased content of coenzyme Q10.Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway.Coenzyme Q supplementation or over-expression of the yeast Coq8 putative kinase stabilizes multi-subunit Coq polypeptide complexes in yeast coq null mutants.Coenzyme Q10 deficiencies in neuromuscular diseases.Glomerular diseases: genetic causes and future therapeutics.Coenzyme Q and mitochondrial disease Mitochondrial dysfunction in the pathophysiology of renal diseases.Genetic bases and clinical manifestations of coenzyme Q10 (CoQ 10) deficiency.Biosynthesis of coenzyme Q in eukaryotes.Biochemical Assessment of Coenzyme Q10 Deficiency.Impact of Chemical Analogs of 4-Hydroxybenzoic Acid on Coenzyme Q Biosynthesis: From Inhibition to Bypass of Coenzyme Q Deficiency.Knockdown of the coenzyme Q synthesis gene Smed-dlp1 affects planarian regeneration and tissue homeostasis.Genetics of coenzyme q10 deficiency.ADCK4 "reenergizes" nephrotic syndrome.Nutritional Interventions for Mitochondrial OXPHOS Deficiencies: Mechanisms and Model Systems.Coenzyme Q10 Supplementation in Aging and Disease.

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P2860

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

http://www.wikidata.org/entity/Q36977423

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AJPRENAL.90445.2008

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American Journal of Physiology - Renal Physiology

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Coenzyme Q10 supplementation r ...... diphosphate synthase subunit 2

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Adam L Lunceford

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Beth Marbois

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Catherine F Clarke

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David L Gasser

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Makoto Kawamukai

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Rhonda King

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Ryoichi Saiki

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Yuchen Shi

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P2860

Characterization of solanesyl and decaprenyl diphosphate synthases in mice and humans

A mutation in para-hydroxybenzoate-polyprenyl transferase (COQ2) causes primary coenzyme Q10 deficiency

CABC1 gene mutations cause ubiquinone deficiency with cerebellar ataxia and seizures

ADCK3, an ancestral kinase, is mutated in a form of recessive ataxia associated with coenzyme Q10 deficiency

Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders

Mechanisms underlying the resistance to diet-induced obesity in germ-free mice

Crystal structure of octaprenyl pyrophosphate synthase from hyperthermophilic Thermotoga maritima and mechanism of product chain length determination

Regulation of product chain length by isoprenyl diphosphate synthases

Thematic review series: lipid posttranslational modifications. Structural biology of protein farnesyltransferase and geranylgeranyltransferase type I

Biochemical functions of coenzyme Q10

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