IDEAS home Printed from https://ideas.repec.org/a/nat/natcom/v13y2022i1d10.1038_s41467-021-27935-9.html
   My bibliography  Save this article

Retinal pigment epithelium-specific CLIC4 mutant is a mouse model of dry age-related macular degeneration

Author

Listed:
  • Jen-Zen Chuang

    (Margaret M. Dyson Vision Research Institute, Weill Cornell Medicine)

  • Nan Yang

    (Margaret M. Dyson Vision Research Institute, Weill Cornell Medicine)

  • Nobuyuki Nakajima

    (Margaret M. Dyson Vision Research Institute, Weill Cornell Medicine
    Tokai University)

  • Wataru Otsu

    (Margaret M. Dyson Vision Research Institute, Weill Cornell Medicine
    Gifu Pharmaceutical University)

  • Cheng Fu

    (Margaret M. Dyson Vision Research Institute, Weill Cornell Medicine)

  • Howard Hua Yang

    (National Cancer Institute, National Institutes of Health)

  • Maxwell Ping Lee

    (National Cancer Institute, National Institutes of Health)

  • Armaan Fazal Akbar

    (National institute of Health)

  • Tudor Constantin Badea

    (National institute of Health
    Transilvania University of Brasov, School of Medicine)

  • Ziqi Guo

    (Margaret M. Dyson Vision Research Institute, Weill Cornell Medicine)

  • Afnan Nuruzzaman

    (Margaret M. Dyson Vision Research Institute, Weill Cornell Medicine)

  • Kuo-Shun Hsu

    (Margaret M. Dyson Vision Research Institute, Weill Cornell Medicine
    Sloan Kettering Cancer Institute)

  • Joshua L. Dunaief

    (University of Pennsylvania)

  • Ching-Hwa Sung

    (Margaret M. Dyson Vision Research Institute, Weill Cornell Medicine
    Weill Cornell Medicine)

Abstract

Age-related macular degeneration (AMD) is the leading cause of blindness among the elderly. Dry AMD has unclear etiology and no treatment. Lipid-rich drusen are the hallmark of dry AMD. An AMD mouse model and insights into drusenogenesis are keys to better understanding of this disease. Chloride intracellular channel 4 (CLIC4) is a pleomorphic protein regulating diverse biological functions. Here we show that retinal pigment epithelium (RPE)-specific Clic4 knockout mice exhibit a full spectrum of functional and pathological hallmarks of dry AMD. Multidisciplinary longitudinal studies of disease progression in these mice support a mechanistic model that links RPE cell-autonomous aberrant lipid metabolism and transport to drusen formation.

Suggested Citation

  • Jen-Zen Chuang & Nan Yang & Nobuyuki Nakajima & Wataru Otsu & Cheng Fu & Howard Hua Yang & Maxwell Ping Lee & Armaan Fazal Akbar & Tudor Constantin Badea & Ziqi Guo & Afnan Nuruzzaman & Kuo-Shun Hsu &, 2022. "Retinal pigment epithelium-specific CLIC4 mutant is a mouse model of dry age-related macular degeneration," Nature Communications, Nature, vol. 13(1), pages 1-14, December.
    • Handle: RePEc:nat:natcom:v:13:y:2022:i:1:d:10.1038_s41467-021-27935-9
    DOI: 10.1038/s41467-021-27935-9
    as

    Download full text from publisher

    File URL: https://www.nature.com/articles/s41467-021-27935-9
    File Function: Abstract
    Download Restriction: no
    File URL: https://libkey.io/10.1038/s41467-021-27935-9?utm_source=ideas
    LibKey link: if access is restricted and if your library uses this service, LibKey will redirect you to where you can use your library subscription to access this item
    ---><---

    More about this item

    Statistics

    Access and download statistics

    Corrections

    All material on this site has been provided by the respective publishers and authors. You can help correct errors and omissions. When requesting a correction, please mention this item's handle: RePEc:nat:natcom:v:13:y:2022:i:1:d:10.1038_s41467-021-27935-9. See general information about how to correct material in RePEc.

    If you have authored this item and are not yet registered with RePEc, we encourage you to do it here. This allows to link your profile to this item. It also allows you to accept potential citations to this item that we are uncertain about.

    We have no bibliographic references for this item. You can help adding them by using this form .

    If you know of missing items citing this one, you can help us creating those links by adding the relevant references in the same way as above, for each refering item. If you are a registered author of this item, you may also want to check the "citations" tab in your RePEc Author Service profile, as there may be some citations waiting for confirmation.

    For technical questions regarding this item, or to correct its authors, title, abstract, bibliographic or download information, contact: Sonal Shukla or Springer Nature Abstracting and Indexing (email available below). General contact details of provider: http://www.nature.com .

    Please note that corrections may take a couple of weeks to filter through the various RePEc services.

    IDEAS is a RePEc service. RePEc uses bibliographic data supplied by the respective publishers.