Retinal Degeneration in Choroideremia follows an Exponential Decay Function
نویسندگان
چکیده
منابع مشابه
Studies of visual function and its decay in mice with hereditary retinal degeneration.
Functional implications of mouse hereditary retinal degeneration have been studied at the level of the superior colliculus and visual cortex in the C57BL/6J-le rd strain. On autoradiography at a light-microscopic level, following eye injection with radioactive compounds, central visual structures appeared normal. A slight reduction in ipsilateral retinal projection was probably related to reduc...
متن کاملIndependent degeneration of photoreceptors and retinal pigment epithelium in conditional knockout mouse models of choroideremia.
Choroideremia (CHM) is an X-linked degeneration of the retinal pigment epithelium (RPE), photoreceptors, and choroid, caused by loss of function of the CHM/REP1 gene. REP1 is involved in lipid modification (prenylation) of Rab GTPases, key regulators of intracellular vesicular transport and organelle dynamics. To study the pathogenesis of CHM and to develop a model for assessing gene therapy, w...
متن کاملRetinal Changes in an ATP-Induced Model of Retinal Degeneration
In rodents and felines, intravitreal administration of adenosine triphosphate (ATP) has been shown to induce photoreceptor death providing a tractable model of retinal degeneration in these species. This study investigated the long term effects of photoreceptor loss in an ATP induced feline model of retinal degeneration. Six normal sighted felines were unilaterally blinded using intravitreal AT...
متن کاملCharacterizing the Natural History of Visual Function in Choroideremia Using Microperimetry and Multimodal Retinal Imaging
Purpose Centripetal retinal degeneration in choroideremia (CHM) leads to early visual field restriction and late central vision loss. The latter marks an acute decline in quality of life but visual prognostication remains challenging. We investigated visual function in CHM by correlating best-corrected visual acuity (BCVA), microperimetry and multimodal imaging. Methods Fifty-six consecutive ...
متن کاملVisual function in myopia without central retinal degeneration.
Is there an additional loss of visual function in myopia beyond that attributable to refractive error and blur of the retinal image? Is there a component ofvisual loss caused by retinal changes? It is well known that in degenerative myopia, the pathological changes include retinal atrophy and may be associated with abnormalities of the electro-retinogram and dark adaptation.1 The paper by Manty...
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ژورنال
عنوان ژورنال: Ophthalmology
سال: 2018
ISSN: 0161-6420
DOI: 10.1016/j.ophtha.2018.02.004