Rescue of primary ubiquinone deficiency due to a novelCOQ7defect using 2,4–dihydroxybensoic acid

Mitochondrial function and lifespan of mice with controlled ubiquinone biosynthesis

(2015) Ying Wang et al.   Nature Communications

Molecular characterization of the human COQ5 C-methyltransferase in coenzyme Q10 biosynthesis

(2014) Theresa P.T. Nguyen et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Ubiquinol-10 ameliorates mitochondrial encephalopathy associated with CoQ deficiency

(2014) Laura García-Corzo et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Rapid pulsed whole genome sequencing for comprehensive acute diagnostics of inborn errors of metabolism

(2014) Henrik Stranneheim et al.   BMC GENOMICS

Mitochondrial COQ9 is a lipid-binding protein that associates with COQ7 to enable coenzyme Q biosynthesis

(2014) D. C. Lohman et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Effect of vanillic acid on COQ6 mutants identified in patients with coenzyme Q10 deficiency

(2013) Mara Doimo et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

The molecular genetics of coenzyme Q biosynthesis in health and disease

(2013) Leila N. Laredj et al.   BIOCHIMIE

ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption

(2013) Shazia Ashraf et al.   JOURNAL OF CLINICAL INVESTIGATION

Overexpression of the Coq8 Kinase inSaccharomyces cerevisiae coqNull Mutants Allows for Accumulation of Diagnostic Intermediates of the Coenzyme Q6Biosynthetic Pathway

(2012) Letian X. Xie et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Coenzyme Q Biosynthesis: Coq6 Is Required for the C5-Hydroxylation Reaction and Substrate Analogs Rescue Coq6 Deficiency

(2011) Mohammad Ozeir et al.   CHEMISTRY & BIOLOGY

COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness

(2011) Saskia F. Heeringa et al.   JOURNAL OF CLINICAL INVESTIGATION

Parkinson's disease-like neuromuscular defects occur in prenyl diphosphate synthase subunit 2 (Pdss2) mutant mice

(2011) Carly G.K. Ziegler et al.   MITOCHONDRION

The Aging-Associated Enzyme CLK-1 Is a Member of the Carboxylate-Bridged Diiron Family of Proteins

(2010) Rachel K. Behan et al.   BIOCHEMISTRY

Treatment of CoQ10 Deficient Fibroblasts with Ubiquinone, CoQ Analogs, and Vitamin C: Time- and Compound-Dependent Effects

(2010) Luis C. López et al.   PLoS One

A Nonsense Mutation in COQ9 Causes Autosomal-Recessive Neonatal-Onset Primary Coenzyme Q10 Deficiency: A Potentially Treatable Form of Mitochondrial Disease

(2009) Andrew J. Duncan et al.   AMERICAN JOURNAL OF HUMAN GENETICS

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

(2008) Min Peng et al.   PLoS Genetics