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Localization of complement 1 inhibitor (C1INH/SERPING1) in human eyes with age-related macular degeneration

Localization of complement 1 inhibitor (C1INH/SERPING1) in human eyes with age-related macular degeneration
Localization of complement 1 inhibitor (C1INH/SERPING1) in human eyes with age-related macular degeneration
Age-related macular degeneration (AMD) is a common degenerative disease resulting in injury to the retina, retinal pigment epithelium and choriocapillaris. Recent data from histopathology, animal models and genetic studies have implicated altered regulation of the complement system as a major factor in the incidence and progression of this disease. A variant in the gene SERPING1, which encodes C1INH, an inhibitor of the classical and lectin pathways of complement activation, was recently shown to be associated with AMD. In this study we sought to determine the localization of C1INH in human donor eyes. Immunofluorescence studies using a monoclonal antibody directed against C1INH revealed localization to photoreceptor cells, inner nuclear layer neurons, choriocapillaris, and choroidal extracellular matrix. Drusen did not exhibit labeling. Genotype at rs2511989 did not appear to affect C1INH abundance or localization, nor was it associated with significant molecular weight differences when evaluated by Western blot. In a small number of eyes (n = 7 AMD and n = 7 control) AMD affection status was correlated with increased abundance of choroidal C1INH. These results indicate that C1INH protein is present in the retina and choroid, where it may regulate complement activation.
macular degeneration, RPE, choroid, retina, complement
0014-4835
767-773
Mullins, Robert F.
794dfee6-5aa1-4e37-93c4-ae5cbce8faf2
Faidley, Elizabeth A.
b59037f6-5c9d-45b4-b4b9-f65946ddf00f
Daggett, Heather T.
b2315951-ff10-409d-acea-9c6e4d20d7cc
Jomary, Catherine
ba6cc4c1-f312-4711-973f-14c95bb9c736
Lotery, Andrew J.
5ecc2d2d-d0b4-468f-ad2c-df7156f8e514
Stone, Edwin M.
545dc2cf-5ba2-4c9d-95aa-e22218c323c5
Mullins, Robert F.
794dfee6-5aa1-4e37-93c4-ae5cbce8faf2
Faidley, Elizabeth A.
b59037f6-5c9d-45b4-b4b9-f65946ddf00f
Daggett, Heather T.
b2315951-ff10-409d-acea-9c6e4d20d7cc
Jomary, Catherine
ba6cc4c1-f312-4711-973f-14c95bb9c736
Lotery, Andrew J.
5ecc2d2d-d0b4-468f-ad2c-df7156f8e514
Stone, Edwin M.
545dc2cf-5ba2-4c9d-95aa-e22218c323c5

Mullins, Robert F., Faidley, Elizabeth A., Daggett, Heather T., Jomary, Catherine, Lotery, Andrew J. and Stone, Edwin M. (2009) Localization of complement 1 inhibitor (C1INH/SERPING1) in human eyes with age-related macular degeneration. Experimental Eye Research, 89 (5), 767-773. (doi:10.1016/j.exer.2009.07.001). (PMID:19607829)

Record type: Article

Abstract

Age-related macular degeneration (AMD) is a common degenerative disease resulting in injury to the retina, retinal pigment epithelium and choriocapillaris. Recent data from histopathology, animal models and genetic studies have implicated altered regulation of the complement system as a major factor in the incidence and progression of this disease. A variant in the gene SERPING1, which encodes C1INH, an inhibitor of the classical and lectin pathways of complement activation, was recently shown to be associated with AMD. In this study we sought to determine the localization of C1INH in human donor eyes. Immunofluorescence studies using a monoclonal antibody directed against C1INH revealed localization to photoreceptor cells, inner nuclear layer neurons, choriocapillaris, and choroidal extracellular matrix. Drusen did not exhibit labeling. Genotype at rs2511989 did not appear to affect C1INH abundance or localization, nor was it associated with significant molecular weight differences when evaluated by Western blot. In a small number of eyes (n = 7 AMD and n = 7 control) AMD affection status was correlated with increased abundance of choroidal C1INH. These results indicate that C1INH protein is present in the retina and choroid, where it may regulate complement activation.

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More information

e-pub ahead of print date: 14 July 2009
Published date: November 2009
Keywords: macular degeneration, RPE, choroid, retina, complement
Organisations: Clinical & Experimental Sciences

Identifiers

Local EPrints ID: 359182
URI: http://eprints.soton.ac.uk/id/eprint/359182
ISSN: 0014-4835
PURE UUID: b5d53205-ea28-4ae8-a565-6f89d80c610c
ORCID for Andrew J. Lotery: ORCID iD orcid.org/0000-0001-5541-4305

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Date deposited: 23 Oct 2013 13:13
Last modified: 15 Mar 2024 03:16

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Contributors

Author: Robert F. Mullins
Author: Elizabeth A. Faidley
Author: Heather T. Daggett
Author: Catherine Jomary
Author: Edwin M. Stone

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