ADCK4 Deficiency Destabilizes the Coenzyme Q Complex, Which Is Rescued by 2,4-Dihydroxybenzoic Acid Treatment

Treatment with 2,4-Dihydroxybenzoic Acid Prevents FSGS Progression and Renal Fibrosis in Podocyte-Specific Coq6 Knockout Mice

(2019) Eugen Widmeier et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Anaerobic Glycolysis Maintains the Glomerular Filtration Barrier Independent of Mitochondrial Metabolism and Dynamics

(2019) Paul T. Brinkkoetter et al.   Cell Reports

Coenzyme Q 10 deficiencies: pathways in yeast and humans

(2018) Agape M. Awad et al.   Essays in Biochemistry

Kaempferol increases levels of coenzyme Q in kidney cells and serves as a biosynthetic ring precursor

(2017) Lucía Fernández-del-Río et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mutations in COQ8B (ADCK4) found in patients with steroid-resistant nephrotic syndrome alter COQ8B function

(2017) Luis Vazquez Fonseca et al.   HUMAN MUTATION

A Personalized Model of COQ2 Nephropathy Rescued by the Wild-Type COQ2 Allele or Dietary Coenzyme Q 10 Supplementation

(2017) Jun-yi Zhu et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Coenzyme Q10 supplementation therapy for 2 children with proteinuria renal disease and ADCK4 mutation

(2017) Chunyue Feng et al.   MEDICINE

Focal segmental glomerulosclerosis and medullary nephrocalcinosis in children with ADCK4 mutations

(2017) Eujin Park et al.   PEDIATRIC NEPHROLOGY

Biochemistry of Mitochondrial Coenzyme Q Biosynthesis

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

A single biochemical activity underlies the pleiotropy of the aging-related protein CLK-1

(2017) Ju-Ling Liu et al.   Scientific Reports

Coenzyme Q biosynthesis in health and disease

(2016) Manuel Jesús Acosta et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Mitochondrial Protein Interaction Mapping Identifies Regulators of Respiratory Chain Function

(2016) Brendan J. Floyd et al.   MOLECULAR CELL

Cerebellar Ataxia and Coenzyme Q Deficiency through Loss of Unorthodox Kinase Activity

(2016) Jonathan A. Stefely et al.   MOLECULAR CELL

ProLuCID: An improved SEQUEST-like algorithm with enhanced sensitivity and specificity

(2015) T. Xu et al.   Journal of Proteomics

ADCK4-Associated Glomerulopathy Causes Adolescence-Onset FSGS

(2015) E. Korkmaz et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Targeted disruption of PDE3B, but not PDE3A, protects murine heart from ischemia/reperfusion injury

(2015) Youn Wook Chung et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Coenzyme Q supplementation or over-expression of the yeast Coq8 putative kinase stabilizes multi-subunit Coq polypeptide complexes in yeast coq null mutants

(2014) Cuiwen H. He et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Ubiad1 Is an Antioxidant Enzyme that Regulates eNOS Activity by CoQ10 Synthesis

(2013) Vera Mugoni et al.   CELL

Podocyte energy metabolism and glomerular diseases

(2013) Toshiyuki Imasawa et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

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

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

Tissue plasminogen activator regulates Purkinje neuron development and survival

(2013) J. Li et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Cell Biology and Pathology of Podocytes

(2011) Anna Greka et al.   Annual Review of Physiology

COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness

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

ADCK3, an Ancestral Kinase, Is Mutated in a Form of Recessive Ataxia Associated with Coenzyme Q10 Deficiency

(2008) Clotilde Lagier-Tourenne et al.   AMERICAN JOURNAL OF HUMAN GENETICS

A Mitochondrial Protein Compendium Elucidates Complex I Disease Biology

(2008) David J. Pagliarini et al.   CELL

Ubiquinone biosynthesis inSaccharomyces cerevisiae: the molecular organization ofO-methylase Coq3p depends on Abc1p/Coq8p

(2008) Anja Tauche et al.   FEMS YEAST RESEARCH