ArticleJournal of the American Society of Nephrology : JASN2020
ADCK4 Deficiency Destabilizes the Coenzyme Q Complex, Which Is Rescued by 2,4-Dihydroxybenzoic Acid Treatment.
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Article in Journal of the American Society of Nephrology : JASN, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers, 2 of them syntheses that pooled it.
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43citing papers in PubMed, 2 pooled it
3.3field-weighted citation impact, top 7% of its field
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Who cites it
43 citing papers in PubMed, 2 syntheses or guidelines pooled it, 59 citations in OpenAlex.
- The efficacy of coenzyme QJournal of cellular and molecular medicine · 2022Pooled it
- Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group.Nature reviews. Nephrology · 2021Guideline
- ADCK1 Regulates Mitochondrial Bioenergetics in Hepatocellular Carcinoma In Vitro.International journal of molecular sciences · 2026Article
- Preclinical and first-in-human evidence of 4-hydroxybenzoic acid for mitochondrial COQ2 deficiency.Brain : a journal of neurology · 2026Article
- Bypass Treatments for Primary Coenzyme Q10 Deficiency: An Update.International journal of molecular sciences · 2026Review
- Integrating Multi-Omics Data to Uncover Causal Links Between Mitochondria-Related Genes and Chronic Obstructive Pulmonary Disease: A Mendelian Randomization Study.International journal of chronic obstructive pulmonary disease · 2026Article
- Functional Testing of Human Disease Missense Variants inKidney international reports · 2025Article
- A Machine Learning Model for the Proteome-Wide Prediction of Lipid-Interacting Proteins.Journal of chemical information and modeling · 2025Article
- The treatment of primary CoQ deficiency requires the targeting of multiple pathogenic mechanisms.Communications medicine · 2025Article
- Therapies for Mitochondrial Disease: Past, Present, and Future.Journal of inherited metabolic disease · 2025Review
- The ADCK Kinase Family: Key Regulators of Bioenergetics and Mitochondrial Function and Their Implications in Human Cancers.International journal of molecular sciences · 2025Review
- Natural Hydroxybenzoic and Hydroxycinnamic Acids Derivatives: Mechanisms of Action and Therapeutic Applications.Antioxidants (Basel, Switzerland) · 2025Review
- Single-cell transcriptomics in a child with coenzyme Q10 nephropathy: potential of single-cell RNA sequencing in pediatric kidney disease.Pediatric nephrology (Berlin, Germany) · 2025Article
- Mechanisms of podocyte injury in genetic kidney disease.Pediatric nephrology (Berlin, Germany) · 2025Review
- Genetics in Kidney Diseases.Advances in kidney disease and health · 2025Review
- Understanding coenzyme Q.Physiological reviews · 2024Review
- β-resorcylic acid released by Limosilactobacillus reuteri protects against cisplatin-induced ovarian toxicity and infertility.Cell reports. Medicine · 2024Article
- Urinary podocyte markers in diabetic kidney disease.Kidney research and clinical practice · 2024Article
- Therapeutic Potential Targeting Podocyte Mitochondrial Dysfunction in Focal Segmental Glomerulosclerosis.Kidney diseases (Basel, Switzerland) · 2023Review
- Biosynthesis, Deficiency, and Supplementation of Coenzyme Q.Antioxidants (Basel, Switzerland) · 2023Review
Corrections and comments
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Authors and funding
17 authors at 4 institutions in 3 countries.
Eugen WidmeierDivision of Nephrology, Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.ORCID 0000-0002-7773-5190
Seyoung YuDepartments of Pharmacology, Yonsei University College of Medicine, Seoul, Korea hygee@yuhs.ac friedhelm.hildebrandt@childrens.harvard.edu.
Anish NagDepartment of Chemistry and Biochemistry, Molecular Biology Institute, University of California, Los Angeles, Los Angeles, California.
Youn Wook ChungSeverance Biomedical Science Institute, Yonsei University College of Medicine, Seoul, Korea.ORCID 0000-0002-4382-1410
Makiko NakayamaDivision of Nephrology, Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
Lucía Fernández-Del-RíoDepartment of Chemistry and Biochemistry, Molecular Biology Institute, University of California, Los Angeles, Los Angeles, California.
Hannah HugoDivision of Nephrology, Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
David SchapiroDivision of Nephrology, Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
Florian BuergerDivision of Nephrology, Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
Won-Il ChoiDivision of Nephrology, Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
Martin HelmstädterRenal Division, Department of Medicine, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Jae-Woo KimBrain Korea 21 PLUS Project for Medical Science, Yonsei University College of Medicine, Seoul, Korea.
Ji-Hwan RyuBrain Korea 21 PLUS Project for Medical Science, Yonsei University College of Medicine, Seoul, Korea.ORCID 0000-0001-6969-7465
Min Goo LeeDepartments of Pharmacology, Yonsei University College of Medicine, Seoul, Korea.
Catherine F ClarkeDepartment of Chemistry and Biochemistry, Molecular Biology Institute, University of California, Los Angeles, Los Angeles, California.
Friedhelm HildebrandtDivision of Nephrology, Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts hygee@yuhs.ac friedhelm.hildebrandt@childrens.harvard.edu.
Heon Yung GeeDepartments of Pharmacology, Yonsei University College of Medicine, Seoul, Korea hygee@yuhs.ac friedhelm.hildebrandt@childrens.harvard.edu.ORCID 0000-0002-8741-6177
Boston Children's Hospital · USYonsei University · KRUniversity of California, Los Angeles · USUniversity of Freiburg · DE
Funding
UAB-UCSD O'Brien Core Center for Acute Kidney Injury ResearchP30DK079337 · NIDDK · UNIVERSITY OF ALABAMA AT BIRMINGHAM · PI AGARWAL, ANUPAM · 2008 to 2022
$18.3MIdentification and function of new genes causing childhood nephrotic syndromeR01DK076683 · NIDDK · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI HILDEBRANDT, FRIEDHELM · 2007 to 2021
$5.3MNIDDK NIH HHS P30 DK079337NIDDK NIH HHS R01 DK076683
Abstract
backgroundMutations in
methodsTo elucidate the ADCK4's function in podocytes, we generated a podocyte-specific,
resultsAbsence of
conclusionsOur study shows that ADCK4 is required for CoQ
Indexed as
AnimalsEnzyme StabilityGlomerulosclerosis, Focal SegmentalHEK293 CellsHumansHydroxybenzoatesMethyltransferasesMiceMice, Inbred C57BLMitochondriaMitochondrial ProteinsPodocytesProtein KinasesUbiquinonebeta-resorcylic acidcoenzyme Q10COQ5 protein, humanCOQ8B protein, humanHydroxybenzoatesMethyltransferasesMitochondrial ProteinsProtein KinasesUbiquinone2,4-dihydroxybenzoic acidADCK4coenzyme Q10Q complexsteroid-resistant nephrotic syndrome
Identifiers
What OpenQuestion holds
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.