Literature DB >> 19892876

Soluble and mature amyloid fibrils in drusen deposits.

J Mario Isas1, Volker Luibl, Lincoln V Johnson, Rakez Kayed, Ronald Wetzel, Charles G Glabe, Ralf Langen, Jeannie Chen.   

Abstract

PURPOSE: Drusen are a hallmark of eyes affected by age-related macular degeneration. In previous study, a conformational-specific antibody showed drusen to contain nonfibrillar amyloid structures. The current study was undertaken to assess the presence of additional amyloid structures in drusen.
METHODS: Sections from human donor eyes were reacted with M204, a monoclonal antibody that recognizes nonfibrillar oligomers; OC, a polyclonal antibody that recognizes amyloid fibrils of various molecular weights; and WO1 and WO2, monoclonal antibodies that are specifically reactive to mature amyloid fibrils. Electron microscopy was used as an independent means of investigating the presence of amyloid fibrils in drusen.
RESULTS: The presence of nonfibrillar oligomers was verified using the M204 antibody. OC and WO antibodies stained a wide spectrum of vesicular structures. OC reactivity showed extensive overlap with Abeta immunoreactivity, whereas a partial overlap was seen between Abeta reactivity and that of the WO antibodies. The presence of amyloid fibrils was also visualized by electron microscopy.
CONCLUSIONS: These data reveal the presence of a wide spectrum of amyloid structures in drusen. The results are significant, given that specific conformational forms of amyloid are known to be pathogenic in a variety of neurodegenerative diseases. Deposition of these structures may lead to local toxicity of the retinal pigmented epithelium or induction of local inflammatory events that contribute to drusen biogenesis and the pathogenesis of AMD.

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Year:  2009        PMID: 19892876      PMCID: PMC2840723          DOI: 10.1167/iovs.09-4207

Source DB:  PubMed          Journal:  Invest Ophthalmol Vis Sci        ISSN: 0146-0404            Impact factor:   4.799


  40 in total

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5.  Structural properties of Abeta protofibrils stabilized by a small molecule.

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6.  Vitronectin is a constituent of ocular drusen and the vitronectin gene is expressed in human retinal pigmented epithelial cells.

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Review 8.  Neurodegenerative diseases: new concepts of pathogenesis and their therapeutic implications.

Authors:  Daniel M Skovronsky; Virginia M-Y Lee; John Q Trojanowski
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9.  Amyloid-beta is found in drusen from some age-related macular degeneration retinas, but not in drusen from normal retinas.

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10.  The Alzheimer's A beta -peptide is deposited at sites of complement activation in pathologic deposits associated with aging and age-related macular degeneration.

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Review 5.  Proteotoxicity and cardiac dysfunction.

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6.  Iron increases APP translation and amyloid-beta production in the retina.

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7.  Near-infrared Fluorescence Ocular Imaging (NIRFOI) of Alzheimer's Disease.

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9.  Inflammatory mediators induced by amyloid-beta in the retina and RPE in vivo: implications for inflammasome activation in age-related macular degeneration.

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10.  NLRP3 inflammasome activation in retinal pigment epithelial cells by lysosomal destabilization: implications for age-related macular degeneration.

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