Abstract:
Purpose:To identify the role of the BBSome protein Bardet-Biedl syndrome 5 (BBS5) in photoreceptor function, protein trafficking, and structure using a congenital mutant mouse model. Methods:Bbs5-/- mice (2 and 9 months old) were used to assess retinal function and morphology. Hematoxylin and eosin staining of retinal sections was performed to visualize histology. Electroretinography was used to analyze rod and cone photoreceptor function. Retinal protein localization was visualized using immunofluorescence (IF) within retinal cryosections. TUNEL staining was used to quantify cell death. Transmission electron microscopy (TEM) was used to examine retinal ultrastructure. Results:In the Bbs5-/- retina, there was a significant loss of nuclei in the outer nuclear layer accompanied by an increase in cell death. Through electroretinography, Bbs5-/- mice showed complete loss of cone photoreceptor function. IF revealed mislocalization of the cone-specific proteins M- and S-opsins, arrestin-4, CNGA3, and GNAT2, as well as a light-dependent arrestin-1 mislocalization, although perpherin-2 was properly localized. TEM revealed abnormal outer segment disk orientation in Bbs5-/-. Conclusions:Collectively, these data suggest that, although BBS5 is a core BBSome component expressed in all ciliated cells, its role within the retina mediates specific photoreceptor protein cargo transport. In the absence of BBS5, cone-specific protein mislocalization and a loss of cone photoreceptor function occur.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Bales KL,Bentley MR,Croyle MJ,Kesterson RA,Yoder BK,Gross AKdoi
10.1167/iovs.61.10.17subject
Has Abstractpub_date
2020-08-03 00:00:00pages
17issue
10eissn
0146-0404issn
1552-5783pii
2770613journal_volume
61pub_type
杂志文章abstract:PURPOSE:meso-Zeaxanthin is a carotenoid that is rarely encountered in nature outside of the vertebrate eye. It is not a constituent of a normal human diet, yet this carotenoid comprises one-third of the primate macular pigment. In the current study, we undertook a systematic approach to biochemically characterize the p...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.16-19111
更新日期:2016-04-01 00:00:00
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journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1999-06-01 00:00:00
abstract:PURPOSE:Retinopathy of prematurity (ROP) is a major problem among very preterm survivors of neonatal intensive care. Neovascularization of the retina is prominent in the proliferative stages of ROP and is under the control of several factors such as vascular endothelial growth factor (VEGF). This study was undertaken o...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.03-1303
更新日期:2004-06-01 00:00:00
abstract::A group of 32 patients with low vision who were considered clinically appropriate candidates for visual rehabilitation with telescopic spectacles were prospectively studied before the first attempted use of these visual aids. Laboratory measurements were made of: (1) rotational head stability in pitch and yaw during q...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1991-09-01 00:00:00
abstract:PURPOSE:To assess the strength of the association between retinal nerve fiber layer (RNFL) thickness and optic disc topography measured with confocal retinal tomography (HRT II; Heidelberg Engineering, Dossenheim, Germany), optical coherence tomography (StratusOCT; Carl Zeiss Meditec, Inc., Dublin, CA), and scanning la...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.05-1489
更新日期:2006-07-01 00:00:00
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journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-15008
更新日期:2015-01-13 00:00:00
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journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-1363
更新日期:2008-06-01 00:00:00
abstract:PURPOSE:Keratoconus is characterized by thinning and scarring of the central region of the cornea. Previous research showed that, in corneas obtained from patients with keratoconus, lysosomal enzyme activities are elevated, whereas levels of protease inhibitors such as alpha1-proteinase inhibitor are reduced. This stud...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1998-06-01 00:00:00
abstract::Topical commercial phenylephrine HCl (Neo-Synephrine 10%) has been shown to cause an increase in corneal thickness and reversible vacuolization of corneal endothelial cells in rabbits. Using an in vivo model of regenerated corneal endothelial cells in the cat, we compared the cytotoxicity of phenylephrine-HCl 10% to r...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1979-03-01 00:00:00
abstract:PURPOSE:To compare the diagnostic value of Bruch's membrane opening minimum rim width (BMO-MRW) and retinal nerve fiber layer thickness (RNFLT) in patients with ocular hypertension, preperimetric, and perimetric glaucoma. METHODS:One hundred eighty-one eyes consisting of 40 healthy controls, 41 ocular hypertensive sub...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章,随机对照试验
doi:10.1167/iovs.15-18906
更新日期:2016-07-01 00:00:00
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journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.11-7210
更新日期:2011-05-01 00:00:00
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journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.04-0147
更新日期:2004-10-01 00:00:00
abstract::Total outflow facility was determined by two-level constant pressure perfusion of the anterior chamber in surgically virgin, aniridic, and ciliary muscle disinserted cynomolgus monkey eyes 6 to 13 times at 1- to 2-month intervals over periods of 8 to 24 months. Facility decreased approximately 15 to 20% between consec...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1983-02-01 00:00:00
abstract:PURPOSE:The aim of this study was to elucidate the molecular mechanisms that lead to a dominant nuclear cataract in a mouse harboring the Y118D mutation in the alphaA-crystallin gene. METHODS:The physicochemical properties of alpha-crystallin obtained from mouse lenses with the Y118D mutation as well as a recombinant ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.08-3070
更新日期:2009-06-01 00:00:00
abstract:PURPOSE:To examine the effects of oxidized low-density lipoproteins (oxLDL) on phagocytosis and processing of photoreceptor outer segments (OS) by retinal pigment epithelial (RPE) cells. METHODS:Confluent cultures of RPE-J cells were pretreated with oxLDL or LDL, and the effects of such treatment on the processing of ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:2001-10-01 00:00:00
abstract:PURPOSE:To determine the molecular mechanisms underlying the protective effects of zinc against oxidative stress in cultured retinal pigment epithelial (RPE) cells. METHODS:Cultured ARPE-19 cells were treated with different concentrations of zinc for various times. Cellular glutathione (GSH) and glutathione disulfide ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.05-1322
更新日期:2006-06-01 00:00:00
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journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.12-10826
更新日期:2012-12-07 00:00:00
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journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章,随机对照试验
doi:10.1167/iovs.09-4807
更新日期:2010-12-01 00:00:00
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journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1987-01-01 00:00:00
abstract:PURPOSE:To quantify the prevalence and effect on visual acuity of macular cysts in a large cohort of patients with retinitis pigmentosa. METHODS:In 316 patients with typical forms of retinitis pigmentosa, visual acuity was measured with Early Treatment Diabetic Retinopathy Study (ETDRS) charts, macular cysts were dete...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.08-1992
更新日期:2008-10-01 00:00:00
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journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1985-04-01 00:00:00
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journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1993-12-01 00:00:00
abstract:PURPOSE:To report neural rim dimensions for South Indians and examine rim shape with relevance to clinical evaluation. METHODS:Healthy phakic participants (n = 623) of the Chennai Glaucoma Study with normal frequency-doubling perimetry underwent complete eye examinations including optic disc digital stereophotography....
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-1210
更新日期:2008-08-01 00:00:00
abstract:PURPOSE:Epidemiologic studies have linked UV-B exposure to development of cortical cataracts, but the underlying molecular mechanism(s) is unresolved. Here, we used a mouse model to examine the nature and distribution of DNA photolesions produced by ocular UV-B irradiation. METHODS:Anesthetized mice, eye globes, or is...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.13-12644
更新日期:2013-10-17 00:00:00
abstract::Isotonic, neutral buffered solutions of benzalkonium chloride or chlorhexidine digluconate were applied topically to one eye of rabbits or human subjects. Contralateral control eyes received phosphate buffered saline as placebo. One-half hour later, the tear film of both eyes was loaded with nonpreserved sodium fluore...
journal_title:Investigative ophthalmology & visual science
pub_type: 临床试验,杂志文章
doi:
更新日期:1984-12-01 00:00:00
abstract:PURPOSE:To investigate the function of connective tissue growth factor (CTGF), a matricellular protein of the CCN (Cyr61/CTGF/Nov) family, in retinal vasculature during development and ischemia. METHODS:CTGF expression was determined using RT-PCR, immunohistochemistry, and transgenic mice carrying CTGF promoter-driven...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.11-7870
更新日期:2011-11-07 00:00:00
abstract:PURPOSE:Dry eye disease (DED) is associated with ocular surface inflammation that is thought to be mediated primarily by CD4 T cells. The purpose of this study was to investigate whether this T cell-mediated immune response is generated in the lymphoid compartment and to characterize the functional phenotype of the T c...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.08-2417
更新日期:2009-08-01 00:00:00
abstract:PURPOSE:The Farnsworth-Munsell 100-Hue test (FM100) is a standardized measure of chromatic discrimination, based on colored cap-sorting, which has been widely used in both adults and children. Its dependence on seriation ability raises questions as to its universal suitability and accuracy in assessing purely sensory d...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-16094
更新日期:2015-05-01 00:00:00
abstract::Growth of the embryonic chick cornea was directly related to, and coordinated with, overall eye growth. During normal development, the size of the embryonic chick cornea increased in three linear phases of diametric growth. Corneal diameter increased at a rate of 216 microns per day between embryonic day 4 (E4) and E7...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1992-11-01 00:00:00
abstract:PURPOSE:Conjunctiva-associated lymphoid tissue (CALT) is assumed to be a key location for the generation of adaptive immune mechanisms of the ocular surface, but functional studies of CALT are still lacking. The purpose of this study was to establish an animal model that enables functional analysis of immune mechanisms...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-0809
更新日期:2008-04-01 00:00:00