Abstract：
:SLC4A11 mutations are associated with Fuchs' endothelial corneal dystrophy (FECD), congenital hereditary endothelial dystrophy (CHED) and Harboyan syndrome (endothelial dystrophy with auditory deficiency). Mice with genetically ablated Slc4a11 recapitulate CHED, exhibiting significant corneal edema and altered endothelial morphology. We recently demonstrated that SLC4A11 functions as an NH3 sensitive, electrogenic H+ transporter. Here, we investigated the properties of five clinically relevant SLC4A11 mutants: R125H, W240S, C386R, V507I and N693A, relative to wild type, expressed in a PS120 fibroblast cell line. The effect of these mutations on the NH4Cl-dependent transporter activity was investigated by intracellular pH and electrophysiology measurements. Relative to plasma membrane expression of NaK ATPase, there were no significant differences in plasma membrane SLC4A11 expression among each mutant and wild type. All mutants revealed a marked decrease in acidification in response to NH4Cl when compared to wild type, indicating a decreased H+ permeability in mutants. All mutants exhibited significantly reduced H+ currents at negative holding potentials as compared to wild type. Uniquely, the C386R and W240S mutants exhibited a different inward current profile upon NH4Cl challenges, suggesting an altered transport mode. Thus, our data suggest that these SLC4A11 mutants, rather than having impaired protein trafficking, show altered H+ flux properties.
journal_name
Exp Eye Resjournal_title
Experimental eye researchauthors
Li S,Hundal KS,Chen X,Choi M,Ogando DG,Obukhov AG,Bonanno JAdoi
10.1016/j.exer.2018.12.003subject
Has Abstractpub_date
2019-03-01 00:00:00pages
86-91eissn
0014-4835issn
1096-0007pii
S0014-4835(18)30515-3journal_volume
180pub_type
杂志文章abstract：:The C-terminal region of peripherin/rds contains three predicted alpha-helical domains. One of these domains, corresponding to amino acids 311-322, form an amphiphilic alpha-helix previously shown to promote membrane fusion. The present studies were conducted to determine how the additional alpha-helical regions of th...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1006/exer.2002.2013
更新日期：2002-08-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1006/exer.1997.0336
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journal_title：Experimental eye research
pub_type： 信件
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更新日期：2010-08-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/s0014-4835(86)80006-9
更新日期：1986-11-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/s0014-4835(85)80017-8
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1006/exer.1997.0444
更新日期：1998-02-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/0014-4835(90)90207-b
更新日期：1990-03-01 00:00:00
abstract：:The causal mechanism of light damage in the albino rat retina has been investigated. Male and female albino Wistar rats weighing 200-300 g and previously maintained in normal room lighting of approximately 15 hr light (0.85 +/- 0.05 X 10(-4) W cm-2) per 9 hr dark cycle were simultaneously exposed continuously to unifo...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/0014-4835(86)90012-6
更新日期：1986-01-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/0014-4835(85)90109-5
更新日期：1985-01-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2014.04.003
更新日期：2014-06-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/0014-4835(83)90104-5
更新日期：1983-05-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章,评审
doi：10.1016/j.exer.2005.01.018
更新日期：2005-06-01 00:00:00
abstract：:Specific [3H]-dexamethasone binding sites were demonstrated in bovine corneal cytosol using titration analyses. Specific glucocorticoid binding to corneal cytosol exhibited a single class of sites with apparent dissociation constants of 4-6 nM and 68-72 fmol binding sites mg-1 cytosolic protein. Association of steroid...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/0014-4835(84)90188-x
更新日期：1984-04-01 00:00:00
abstract：:The CLN2 form of neuronal ceroid lipofuscinosis is an autosomal recessively inherited lysosomal storage disease that is characterized by progressive vision loss culminating in blindness, cognitive and motor decline, neurodegeneration, and premature death. CLN2 disease results from mutations in the gene that encodes th...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2015.02.012
更新日期：2015-05-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2007.01.022
更新日期：2007-05-01 00:00:00
abstract：:A number of variants of the wild-type (wt) estrogen receptor beta (ERbeta-1) coexist in a wide range of tissues. In the human these include, together with others, the expression of several isoforms (ERbeta-2-ERbeta-5) due to alternative splicing of exons encoding the carboxy terminus. In this study, we determined whet...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2005.01.019
更新日期：2005-08-01 00:00:00
abstract：:Autoradiographic studies showed selective accumulation of [3H]-glycine, [3H]-GABA, and the GABA agonist [3H]-muscimol by neurons of the mudpuppy retina. [3H]-Glycine was taken up by bipolar cells, amacrine cells, and displaced amacrine or ganglion cells. Both [3H]-GABA and [3H]-muscimol were also accumulated by bipola...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/0014-4835(84)90116-7
更新日期：1984-07-01 00:00:00
abstract：:Human retinal pigment epithelial cells in culture demonstrate adenylate cyclase activity. It is membrane-bound and modulated by GTP regulatory proteins. It is effectively activated only by beta-adrenergic agonists (L-isoproterenol greater than or equal to L-epinephrine greater than L-norepinephrine) and some prostagla...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/s0014-4835(87)80158-6
更新日期：1987-04-01 00:00:00
abstract：:The wound-healing response is critical to the outcome of refractive surgery and studying wound healing contributes to an understanding of the pathophysiology of other corneal injuries. Animal models allow research to be conducted with sufficient samples and under controlled parameters. We studied the hen to determine ...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2006.02.017
更新日期：2006-10-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
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更新日期：1992-04-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
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journal_title：Experimental eye research
pub_type： 杂志文章
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更新日期：2010-12-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/0014-4835(91)90257-f
更新日期：1991-02-01 00:00:00
abstract：:Accurate thermal models for the cornea of the eye support the development of thermal techniques for reshaping the cornea and other scientific purposes. Heat transfer in the cornea must be quantified accurately so that a thermal treatment does not destroy the endothelial layer, which cannot regenerate, and yet is respo...
journal_title：Experimental eye research
pub_type： 杂志文章
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更新日期：2013-10-01 00:00:00
abstract：:Ocular rigidity (OR) is thought to play a role in the pathogenesis of glaucoma, but the lack of reliable non-invasive measurements has been a major technical challenge. We recently developed a clinical method using optical coherence tomography time-lapse imaging and automated choroidal segmentation to measure the puls...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2019.107831
更新日期：2020-01-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2005.10.009
更新日期：2006-06-01 00:00:00
abstract：:Endostatin is an endogenous angiogenesis inhibitor which requires E-selectin for its antiangiogenic activity. The aim of this study was to investigate the expression of endostatin in human choroidal neovascular membranes (CNV) secondary to age-related macular degeneration (AMD) with regard to vascularization and proli...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2005.12.017
更新日期：2006-08-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2020.108367
更新日期：2021-01-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2008.02.001
更新日期：2008-05-01 00:00:00
abstract：:Lacritin is a mitogen of human salivary gland cells as well as a stimulator of human corneal epithelial cells. It is expected to be an important factor in maintaining the surrounding ocular surface. The monkey would be a relevant animal model in which to study the role of lacritin in ophthalmic physiology and patholog...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2007.07.019
更新日期：2007-11-01 00:00:00
