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H. Vorum, M. Ostergaard, B. Honoré, T. Bek; Identification of Differentially Regulated Proteins in Leber's Congenital Amaurosis – A Proteomic Study . Invest. Ophthalmol. Vis. Sci. 2005;46(13):5177.
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© ARVO (1962-2015); The Authors (2016-present)
Purpose: To identify the pattern of protein expression in the retina in Leber's Congenital Amaurosis (LCA). Methods: The retina and sclera from one eye of a patient with LCA and a control eye were studied. The tissue was subjected to high resolution two–dimensional gel electrophoresis, and after development of individual protein spots by silver staining, the expression profile of the pattern of proteins was identified by computerized image analysis. The spots that differed between tissue from the LCA patient and the control person were excised and analyzed by mass spectrometry. Results: There was no difference in the scleral protein expression profile between the patient with LCA and tissue from the control eye. However, in the retina seven proteins were upregulated in the LCA retina, of which five could be identified as: Glutathione–S–transferase, triosephophate isomerase, alphaA–crystallin, subunit a of ATP–synthase and a fragment of retinoblastoma associated protein (RAP140). A protein spot which was downregulated in the LCA retina was identified as a fragment of tubulin beta–1. Conclusions: Retinal tissue in Leber's congenital amaurosis has an upregulation of proteins involved in detoxification, glycolysis, cytoskeleton formation, oxidative phosphorylation, and oncogenesis. These proteins may play a crucial role for the retinal degeneration processes in LCA and other retinal dystrophies.
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