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Short-Wavelength Sensitive Cone (S-cone) Testing as an Outcome Measure for NR2E3 Clinical Treatment Trials.

Abstract：

:Recessively-inherited NR2E3 gene mutations cause an unusual retinopathy with abnormally-increased short-wavelength sensitive cone (S-cone) function, in addition to reduced rod and long/middle-wavelength sensitive cone (L/M-cone) function. Progress toward clinical trials to treat patients with this otherwise incurable retinal degeneration prompted the need to determine efficacy outcome measures. Comparisons were made between three computerized perimeters available in the clinic. These perimeters could deliver short-wavelength stimuli on longer-wavelength adapting backgrounds to measure whether S-cone vision can be quantified. Results from a cohort of normal subjects were compared across the three perimeters to determine S-cone isolation and test-retest variability. S-cone perimetry data from NR2E3-ESCS (enhanced S-cone syndrome) patients were examined and determined to have five stages of disease severity. Using these stages, strategies were proposed for monitoring efficacy of either a focal or retina-wide intervention. This work sets the stage for clinical trials.

journal_name

Int J Mol Sci

journal_title

International journal of molecular sciences

authors

Roman AJ,Powers CA,Semenov EP,Sheplock R,Aksianiuk V,Russell RC,Sumaroka A,Garafalo AV,Cideciyan AV,Jacobson SG

doi

10.3390/ijms20102497

subject

Has Abstract

pub_date

2019-05-21 00:00:00

issue

10

issn

1422-0067

pii

ijms20102497

journal_volume

20

pub_type

杂志文章

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