Disruption of the human cystin-1 myristoyl-electrostatic switch causes polycystic kidney disease that phenocopies autosomal recessive polycystic kidney disease.

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Title: Disruption of the human cystin-1 myristoyl-electrostatic switch causes polycystic kidney disease that phenocopies autosomal recessive polycystic kidney disease.
Authors: Yang C; Center for Translational Science, Children's National Research Institute, Children's National Hospital, Washington, District of Columbia, USA; Center for Precision Medicine and Genomics Research, Children's National Research Institute, Children's National Hospital, Washington, District of Columbia, USA., Harafuji N; Center for Translational Science, Children's National Research Institute, Children's National Hospital, Washington, District of Columbia, USA; Center for Precision Medicine and Genomics Research, Children's National Research Institute, Children's National Hospital, Washington, District of Columbia, USA., Watts JA; Center for Translational Science, Children's National Research Institute, Children's National Hospital, Washington, District of Columbia, USA., Tao B; Department of Cell, Developmental, and Integrative Biology, University of Alabama at Birmingham, Birmingham, Alabama, USA., Moran C; Integrated Biomedical Sciences Program, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA., Clements J; Integrated Biomedical Sciences Program, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA., Price K; Integrated Biomedical Sciences Program, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA., Laucevicius A; Integrated Biomedical Sciences Program, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA., Burrill N; Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA., Gebb J; Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA., Soni S; Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA., Oliver E; Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA., Savla JJ; Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA., Christ L; Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA., Moldenhauer J; Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA., Hartung EA; Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA., Didier R; Department of Radiology, Boston Children's Hospital, Boston, Massachusetts, USA., Santani A; Veritas Genetics, Danvers, Massachusetts, USA., Sandford RN; Academic Department of Genomic Medicine, University of Cambridge, Cambridge, UK., Selkirk L; Luton and Dunstable University Hospital, Lutton, UK., Radley JA; North West Thames Regional Genetics Service, London, UK., Mann K; East Genomic Laboratory Hub, Cambridge University Hospitals National Health Service Foundation Trust, Cambridge, UK., Simonicova I; East Genomic Laboratory Hub, Cambridge University Hospitals National Health Service Foundation Trust, Cambridge, UK., Karl R; Institute of Innate Immunity, Medical Faculty, University of Bonn, Bonn, Germany., Kariat Ashraf AP; Institute of Innate Immunity, Medical Faculty, University of Bonn, Bonn, Germany., Wachten D; Institute of Innate Immunity, Medical Faculty, University of Bonn, Bonn, Germany., Wilson L; Department of Pharmacology and Toxicology, University of Alabama at Birmingham, Birmingham, Alabama, USA; Targeted Metabolomics and Proteomics Laboratory, University of Alabama at Birmingham, Birmingham, Alabama, USA., Bebok Z; Department of Cell, Developmental, and Integrative Biology, University of Alabama at Birmingham, Birmingham, Alabama, USA., Caldovic L; Center for Precision Medicine and Genomics Research, Children's National Research Institute, Children's National Hospital, Washington, District of Columbia, USA; Department of Biochemistry and Molecular Medicine, School of Medicine and Health Sciences, The George Washington University, Washington, District of Columbia, USA., Guay-Woodford LM; Center for Translational Science, Children's National Research Institute, Children's National Hospital, Washington, District of Columbia, USA; Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. Electronic address: guaywoodfl@chop.edu.
Source: Kidney international [Kidney Int] 2026 May; Vol. 109 (5), pp. 939-956. Date of Electronic Publication: 2026 Feb 18.
Publication Type: Journal Article
Journal Info: Publisher: Elsevier Country of Publication: United States NLM ID: 0323470 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1523-1755 (Electronic) Linking ISSN: 00852538 NLM ISO Abbreviation: Kidney Int Subsets: MEDLINE
Database: MEDLINE Ultimate
Description
ISSN:1523-1755
DOI:10.1016/j.kint.2026.01.023