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Identification of IFRD1 as a modifier gene for cystic fibrosis lung disease

Author

Listed:
  • YuanYuan Gu

    (Division of Molecular Immunology,)

  • Isaac T. W. Harley

    (Division of Molecular Immunology,)

  • Lindsay B. Henderson

    (McKusick-Nathans Institute of Genetic Medicine, and,)

  • Bruce J. Aronow

    (Division of Biomedical Informatics,)

  • Ilja Vietor

    (Biocenter, Innsbruck Medical University)

  • Lukas A. Huber

    (Biocenter, Innsbruck Medical University)

  • John B. Harley

    (Arthritis & Immunology Program, Oklahoma Medical Research Foundation, and JK Autoimmunity Inc., Oklahoma City, Oklahoma 73104, USA)

  • Jeffrey R. Kilpatrick

    (Arthritis & Immunology Program, Oklahoma Medical Research Foundation, and JK Autoimmunity Inc., Oklahoma City, Oklahoma 73104, USA)

  • Carl D. Langefeld

    (Wake Forest University School of Medicine, Winston-Salem, North Carolina 27157, USA)

  • Adrienne H. Williams

    (Wake Forest University School of Medicine, Winston-Salem, North Carolina 27157, USA)

  • Anil G. Jegga

    (Division of Biomedical Informatics,)

  • Jing Chen

    (Division of Biomedical Informatics,)

  • Marsha Wills-Karp

    (and)

  • S. Hasan Arshad

    (The David Hide Asthma and Allergy Research Centre, Newport, Isle of Wight, PO30 5TG, UK)

  • Susan L. Ewart

    (College of Veterinary Medicine, Michigan State University, East Lansing, Michigan 48824, USA)

  • Chloe L. Thio

    (Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA)

  • Leah M. Flick

    (Division of Molecular Immunology,)

  • Marie-Dominique Filippi

    (Cincinnati Children’s Hospital Research Foundation and the University of Cincinnati College of Medicine, Cincinnati, Ohio 45229, USA)

  • H. Leighton Grimes

    (and)

  • Mitchell L. Drumm

    (Case Western Reserve University, Cleveland, Ohio 44106, USA)

  • Garry R. Cutting

    (McKusick-Nathans Institute of Genetic Medicine, and,)

  • Michael R. Knowles

    (Cystic Fibrosis–Pulmonary Research and Treatment Center, University of North Carolina, Chapel Hill, North Carolina 27599, USA)

  • Christopher L. Karp

    (Division of Molecular Immunology,)

Abstract

Cystic fibrosis: susceptibility to lung disease The severity of lung disease in patients with cystic fibrosis varies markedly between individuals, and has been shown to have considerable heritability, independent of the genotype of the CFTR (cystic fibrosis transmembrane regulator) mutation that causes the primary disease. Genomic analysis of more than 300 cystic fibrosis patients has led to the identification of the neutrophil transcriptional co-regulator IFRD1 as a genetic modifier for lung disease, acting via an effect on neutrophil effectors. This finding highlights IFRD1 as a possible drug target in cystic fibrosis, of potential clinical significance because lung disease is the major cause of morbidity and mortality in the condition.

Suggested Citation

  • YuanYuan Gu & Isaac T. W. Harley & Lindsay B. Henderson & Bruce J. Aronow & Ilja Vietor & Lukas A. Huber & John B. Harley & Jeffrey R. Kilpatrick & Carl D. Langefeld & Adrienne H. Williams & Anil G. J, 2009. "Identification of IFRD1 as a modifier gene for cystic fibrosis lung disease," Nature, Nature, vol. 458(7241), pages 1039-1042, April.
    • Handle: RePEc:nat:nature:v:458:y:2009:i:7241:d:10.1038_nature07811
    DOI: 10.1038/nature07811
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