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Endo-Lysosome Function in the Retinal Pigment Epithelium in Health and Disease

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Retinal Degenerative Diseases

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 723))

Abstract

Polarized cells such as the retinal pigment epithelium (RPE) have elaborate endo-lysosomal networks that enable them to carry out efficient trafficking and metabolism of proteins and lipids, which is crucial for maintaining cellular homeostasis. Once thought to be little more than garbage disposal systems, late endosomes and lysosomes are now being recognized as highly dynamic platforms for macromolecule sorting, signaling, and cholesterol metabolism. Aging and age-related diseases are characterized by decreased endo-lysosomal function, disturbed cholesterol metabolism, and increased accumulation of lipid–protein aggregates called lipofuscin in postmitotic cells such as neurons and the RPE. Cholesterol accumulation and impaired endo-lysosomal function have emerged as key players in Alzheimer’s and Parkinson’s diseases. This review focuses on recent data supporting a link between endo-lysosome dysfunction, lipofuscin accumulation, and abnormal cholesterol homeostasis in the pathogenesis of age-related macular degeneration (AMD).

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Acknowledgments

Supported by grants from the American Health Assistance Foundation (M2009093) and the Karl Kirchgessner Foundation and a career development award from Research to Prevent Blindness

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Authors and Affiliations

  1. Department of Ophthalmology and Visual Sciences, School of Medicine and Public Health, University of Wisconsin-Madison, 1300 University Ave, MSC 3385, Madison, WI, 53706, USA

    Aparna Lakkaraju

Authors
  1. Aparna Lakkaraju
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Correspondence to Aparna Lakkaraju .

Editor information

Editors and Affiliations

  1. University of California, San Francisco, n/a, San Francisco, 94143, USA

    Matthew M. LaVail

  2. University of Oklahoma Health Sciences C, Oklahoma City, 73104, USA

    John D. Ash

  3. Health Science Center, Dean A. McGee Eye Inst., University of Oklahoma, Stanton L. Young Blvd. 608, OKLAHOMA CITY, 73104, Oklahoma, USA

    Robert E. Anderson

  4. Division of Ophthalmology, Cole Eye Institute at the Cleveland Clin, Euclid Ave. 9500, Cleveland, 44195, Ohio, USA

    Joe G. Hollyfield

  5. Experimental Ophthalmology, University of Zurich, ZURICH, 8091, Switzerland

    Christian Grimm

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© 2012 Springer Science+Business Media, LLC

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Lakkaraju, A. (2012). Endo-Lysosome Function in the Retinal Pigment Epithelium in Health and Disease. In: LaVail, M., Ash, J., Anderson, R., Hollyfield, J., Grimm, C. (eds) Retinal Degenerative Diseases. Advances in Experimental Medicine and Biology, vol 723. Springer, Boston, MA. https://doi.org/10.1007/978-1-4614-0631-0_92

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