Beyond the cherry-red spot: Ocular manifestations of sphingolipid-mediated neurodegenerative and inflammatory disorders

Surv Ophthalmol. 2014 Jan-Feb;59(1):64-76. doi: 10.1016/j.survophthal.2013.02.005. Epub 2013 Sep 5.

Abstract

Sphingolipids are a ubiquitous membrane lipid present in every cell and found most abundantly in neural tissues. Disorders such as Tay-Sachs or Niemann-Pick disease are the most familiar examples of dysfunction in sphingolipid metabolism and are typically associated with neurodegeneration and ocular findings such as blindness. More recently, the role of bioactive sphingolipids has been established in a multitude of cellular events, including cell survival, growth, senescence and apoptosis, inflammation, and neovascularization. We discuss our current knowledge and understanding of sphingolipid metabolism and signaling in the pathogenesis of ocular diseases.

Keywords: FTY720; ceramide; retina; retinal degenerations; sphingolipid; sphingosine; uveitis.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / metabolism
  • Humans
  • Optic Nerve Diseases / diagnosis*
  • Optic Nerve Diseases / metabolism
  • Retinal Degeneration / diagnosis*
  • Retinal Degeneration / metabolism
  • Sphingolipidoses / diagnosis*
  • Sphingolipidoses / metabolism
  • Sphingolipids / metabolism
  • Uveitis, Posterior / diagnosis*
  • Uveitis, Posterior / metabolism

Substances

  • Sphingolipids