Retinal Degenerative Diseases

  • Sparrow J
  • Yamamoto K
  • Ablonczy Z
 et al. 
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Lipofuscin accumulates in the aging retinal pigment epithelium (RPE). It is identified by its fluorescence; however, lipofuscin is a complex mixture, and fluorescence is not specific enough to identify its individual components. Utilizing matrix-assisted laser desorption-ionization imaging, we have recently determined the spatial distribution of lipofuscin components across the RPE. One of the most abundant signals was that of the bis -retinoid A2E, a byproduct of the visual cycle. To better understand the accumulation of A2E, we studied wild-type ( wt ), Rpe65 −/− , and Abca4 −/− mice. A2E was not found in Rpe65 −/− animals. In wt animals, A2E was most abundant in the center of the RPE and diminished toward the periphery. In contrast, the A2E signal was more intense and uniformly distributed in Abca4 −/− mice. The oxidized forms of A2E were also spatially localized. Furthermore, a highly sensitive liquid chromatography–tandem mass spectrometry (LC-MS/MS) method was utilized to quantitate A2E. A2E oxidation sites were determined both in organic extracts and directly from the tissue. The ability to image a specific retinoid and its modified products from fresh tissue suggests wide applicability in the research and potential treatments of retinal degeneration.

Author-supplied keywords

  • Biomedical and Life Sciences
  • a2e
  • abca4
  • age-related macular
  • age-related macular degeneration
  • apolipoprotein
  • autoimmunity
  • biomarkers
  • bisretinoids
  • cholesterol
  • complement system
  • elovl-4
  • hyperoxia
  • infl ammation
  • late endosome
  • light damage
  • lipofuscin
  • lysosome
  • macular degeneration
  • maldi imaging
  • mass spectrometry
  • niemann
  • p23h
  • pathogenesis
  • photooxidation
  • pick c1
  • protein lipid
  • quantitation
  • retina
  • retinal degeneration
  • retinal pigment epithelium
  • stargardt disease
  • traffi cking
  • vitamin a aldehyde

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  • Janet R Sparrow

  • Kazunori Yamamoto

  • Zsolt Ablonczy

  • Danielle B Gutierrez

  • Angus C Grey

  • Kevin L Schey

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