Cystatin C in macular and neuronal degenerations: implications for mechanism(s) of age-related macular degeneration

Luminita Paraoan, Paul Hiscott, Christine Gosden, Ian Grierson

Research output: Contribution to journalArticle (journal)peer-review

29 Citations (Scopus)

Abstract

Cystatin C is a strong inhibitor of cysteine proteinases expressed by diverse cells. Variant B cystatin C, which was associated with increased risk of developing age-related macular degeneration, differs from the wild type protein by a single amino acid (A25T) in the signal sequence responsible for its targeting to the secretory pathway. The same variant conveys susceptibility to Alzheimer disease. Our investigations of the trafficking and processing of variant B cystatin C in living RPE cells highlight impaired secretion of extracellular modulators and inappropriate protein retention in RPE cells as potential molecular mechanisms underpinning macular, and possibly neuronal, degeneration.

Original languageEnglish
Pages (from-to)737-42
Number of pages6
JournalVision Research
Volume50
Issue number7
DOIs
Publication statusPublished - 31 Mar 2010
Externally publishedYes

Keywords

  • Cystatin C/chemistry
  • Eye/chemistry
  • Humans
  • Immunohistochemistry
  • Macular Degeneration/immunology
  • Pigment Epithelium of Eye/metabolism
  • Retinal Neurons/metabolism

Fingerprint

Dive into the research topics of 'Cystatin C in macular and neuronal degenerations: implications for mechanism(s) of age-related macular degeneration'. Together they form a unique fingerprint.

Cite this