Vanillic Acid Restores Coenzyme Q Biosynthesis and ATP Production in Human Cells Lacking COQ6

The idebenone metabolite QS10 restores electron transfer in complex I and coenzyme Q defects

(2018) Valentina Giorgio et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

COX16 is required for assembly of cytochrome c oxidase in human cells and is involved in copper delivery to COX2

(2018) Cristina Cerqua et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Clinical syndromes associated with Coenzyme Q 10 deficiency

(2018) María Alcázar-Fabra et al.   Essays in Biochemistry

Bypassing human CoQ 10 deficiency

(2018) Diran Herebian et al.   MOLECULAR GENETICS AND METABOLISM

Molecular and cellular basis of ornithine δ-aminotransferase deficiency caused by the V332M mutation associated with gyrate atrophy of the choroid and retina

(2018) Riccardo Montioli et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

β‐RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9R239X mice

(2018) Agustín Hidalgo‐Gutiérrez et al.   EMBO Molecular Medicine

Pathogenicity of two COQ7 mutations and responses to 2,4-dihydroxybenzoate bypass treatment

(2017) Ying Wang et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Biochemistry of Mitochondrial Coenzyme Q Biosynthesis

(2017) Jonathan A. Stefely et al.   TRENDS IN BIOCHEMICAL SCIENCES

Impact of Chemical Analogs of 4-Hydroxybenzoic Acid on Coenzyme Q Biosynthesis: From Inhibition to Bypass of Coenzyme Q Deficiency

(2017) Fabien Pierrel   Frontiers in Physiology

TheCOQ2genotype predicts the severity of coenzyme Q10deficiency

(2016) Maria Andrea Desbats et al.   HUMAN MOLECULAR GENETICS

Mitochondrial protein functions elucidated by multi-omic mass spectrometry profiling

(2016) Jonathan A Stefely et al.   NATURE BIOTECHNOLOGY

Biosynthesis of coenzyme Q in eukaryotes

(2015) Makoto Kawamukai   BIOSCIENCE BIOTECHNOLOGY AND BIOCHEMISTRY

Coq6 Is Responsible for the C4-deamination Reaction in Coenzyme Q Biosynthesis inSaccharomyces cerevisiae

(2015) Mohammad Ozeir et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

(2015) Christoph Freyer et al.   JOURNAL OF MEDICAL GENETICS

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

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

Restoring de novo coenzyme Q biosynthesis in Caenorhabditis elegans coq-3 mutants yields profound rescue compared to exogenous coenzyme Q supplementation

(2012) Fernando Gomez et al.   GENE

Haploinsufficiency ofCOQ4causes coenzyme Q10deficiency

(2012) Leonardo Salviati et al.   JOURNAL OF MEDICAL GENETICS

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

(2011) Mohammad Ozeir et al.   CHEMISTRY & BIOLOGY

Probucol ameliorates renal and metabolic sequelae of primary CoQ deficiency inPdss2mutant mice

(2011) Marni J. Falk et al.   EMBO Molecular Medicine

COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness

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

Optimization of respiratory chain enzymatic assays in muscle for the diagnosis of mitochondrial disorders

(2011) Marco Spinazzi et al.   MITOCHONDRION

The food additive vanillic acid controls transgene expression in mammalian cells and mice

(2011) Marc Gitzinger et al.   NUCLEIC ACIDS RESEARCH

Reactive oxygen species, oxidative stress, and cell death correlate with level of CoQ10 deficiency

(2010) Catarina M. Quinzii et al.   FASEB JOURNAL

Functional characterization of human COQ4, a gene required for Coenzyme Q10 biosynthesis

(2008) Alberto Casarin et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

The yeast Coq4 polypeptide organizes a mitochondrial protein complex essential for coenzyme Q biosynthesis

(2008) Beth Marbois et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS