Literature DB >> 19421767

Bestrophin detected in the basal membrane of the retinal epithelium and drusen of monkeys with drusenoid maculopathy.

P Gouras1, K Braun, L Ivert, M Neuringer, J A Mattison.   

Abstract

PURPOSE: To determine if bestrophin is present in the basal membrane of macular retinal pigment epithelium (RPE) and in drusen of rhesus monkeys with age-related drusenoid maculopathy.
METHODS: The macular region of three rhesus monkeys (Macaca mulatta), 23-24 years of age, with drusenoid maculopathy was dissected from eyes fixed with 4% paraformaldehyde. The macula was sectioned into rectangular pieces. The sclera was removed from each segment and the remainder separated into segments of neural retina with retinal epithelium or choroid with retinal epithelium. These segments were incubated with a goat polyclonal antibody to human bestrophin 1, reacted with gold-labeled rabbit antibody to goat IgG, silver-enhanced, and processed for transmission electron microscopy.
RESULTS: Bestrophin-labeled gold particles were found in quasi-linear arrays on the basal surface of the macular RPE and also within drusen where bestrophin was found in segments of membranous-like material. The array density of the bestrophin-linked gold particles on the basal membrane of the epithelium had a maximal value of about 5-100 bestrophin molecules/micron(2). Immuno-detection of bestrophin was most effective when examined in an RPE layer that remained attached to the neural retina, where the basal surface of the epithelium is more directly exposed to the antibodies.
CONCLUSION: Bestrophin is present on the basal membrane of macular RPE of rhesus monkeys with age-related drusenoid maculopathy, and also found in the membranous-like structures of drusen. The latter finding provides insight into the pathogenesis of drusen by indicating that segments of the basal membrane of RPE contribute to the material that accumulates within drusen.

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Year:  2009        PMID: 19421767      PMCID: PMC2752852          DOI: 10.1007/s00417-009-1091-9

Source DB:  PubMed          Journal:  Graefes Arch Clin Exp Ophthalmol        ISSN: 0721-832X            Impact factor:   3.117


  9 in total

1.  Bestrophin, the product of the Best vitelliform macular dystrophy gene (VMD2), localizes to the basolateral plasma membrane of the retinal pigment epithelium.

Authors:  A D Marmorstein; L Y Marmorstein; M Rayborn; X Wang; J G Hollyfield; K Petrukhin
Journal:  Proc Natl Acad Sci U S A       Date:  2000-11-07       Impact factor: 11.205

2.  Pathogenesis of drusen in the primate.

Authors:  T Ishibashi; N Sorgente; R Patterson; S J Ryan
Journal:  Invest Ophthalmol Vis Sci       Date:  1986-02       Impact factor: 4.799

3.  Identification of the gene responsible for Best macular dystrophy.

Authors:  K Petrukhin; M J Koisti; B Bakall; W Li; G Xie; T Marknell; O Sandgren; K Forsman; G Holmgren; S Andreasson; M Vujic; A A Bergen; V McGarty-Dugan; D Figueroa; C P Austin; M L Metzker; C T Caskey; C Wadelius
Journal:  Nat Genet       Date:  1998-07       Impact factor: 38.330

4.  Clinico-morphologic correlations of drusen of Bruch's membrane.

Authors:  R P Burns; L Feeney-Burns
Journal:  Trans Am Ophthalmol Soc       Date:  1980

5.  Mutations in a novel gene, VMD2, encoding a protein of unknown properties cause juvenile-onset vitelliform macular dystrophy (Best's disease).

Authors:  A Marquardt; H Stöhr; L A Passmore; F Krämer; A Rivera; B H Weber
Journal:  Hum Mol Genet       Date:  1998-09       Impact factor: 6.150

6.  Drusenoid maculopathy in rhesus monkeys: autofluorescence, lipofuscin and drusen pathogenesis.

Authors:  Peter Gouras; Lena Ivert; Julie A Mattison; Donald K Ingram; Martha Neuringer
Journal:  Graefes Arch Clin Exp Ophthalmol       Date:  2008-08-12       Impact factor: 3.117

7.  Drusenoid maculopathy in rhesus monkeys (Macaca mulatta): effects of age and gender.

Authors:  Peter Gouras; Lena Ivert; Noelle Landauer; Julie A Mattison; Donald K Ingram; Martha Neuringer
Journal:  Graefes Arch Clin Exp Ophthalmol       Date:  2008-08-16       Impact factor: 3.117

8.  Differential macular and peripheral expression of bestrophin in human eyes and its implication for best disease.

Authors:  Robert F Mullins; Markus H Kuehn; Elizabeth A Faidley; Nasreen A Syed; Edwin M Stone
Journal:  Invest Ophthalmol Vis Sci       Date:  2007-07       Impact factor: 4.799

Review 9.  Molecular physiology of bestrophins: multifunctional membrane proteins linked to best disease and other retinopathies.

Authors:  H Criss Hartzell; Zhiqiang Qu; Kuai Yu; Qinghuan Xiao; Li-Ting Chien
Journal:  Physiol Rev       Date:  2008-04       Impact factor: 37.312

  9 in total
  6 in total

Review 1.  Bestrophins and retinopathies.

Authors:  Qinghuan Xiao; H Criss Hartzell; Kuai Yu
Journal:  Pflugers Arch       Date:  2010-03-28       Impact factor: 3.657

Review 2.  Bestrophin 1 and retinal disease.

Authors:  Adiv A Johnson; Karina E Guziewicz; C Justin Lee; Ravi C Kalathur; Jose S Pulido; Lihua Y Marmorstein; Alan D Marmorstein
Journal:  Prog Retin Eye Res       Date:  2017-01-30       Impact factor: 21.198

3.  Retinal pigment epithelial expression of complement regulator CD46 is altered early in the course of geographic atrophy.

Authors:  Susan D Vogt; Christine A Curcio; Lan Wang; Chuan-Ming Li; Gerald McGwin; Nancy E Medeiros; Nancy J Philp; James A Kimble; Russell W Read
Journal:  Exp Eye Res       Date:  2011-06-12       Impact factor: 3.467

4.  Abundant lipid and protein components of drusen.

Authors:  Lan Wang; Mark E Clark; David K Crossman; Kyoko Kojima; Jeffrey D Messinger; James A Mobley; Christine A Curcio
Journal:  PLoS One       Date:  2010-04-23       Impact factor: 3.240

5.  Disease-causing mutations associated with four bestrophinopathies exhibit disparate effects on the localization, but not the oligomerization, of Bestrophin-1.

Authors:  Adiv A Johnson; Yong-Suk Lee; Andrew J Chadburn; Paolo Tammaro; Forbes D Manson; Lihua Y Marmorstein; Alan D Marmorstein
Journal:  Exp Eye Res       Date:  2014-02-19       Impact factor: 3.467

6.  Bestrophin-1 influences transepithelial electrical properties and Ca2+ signaling in human retinal pigment epithelium.

Authors:  Alan D Marmorstein; Tyson R Kinnick; J Brett Stanton; Adiv A Johnson; Ronald M Lynch; Lihua Y Marmorstein
Journal:  Mol Vis       Date:  2015-04-01       Impact factor: 2.367

  6 in total

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