Cuenca Nicolás, Fernández-Sánchez Laura, Sauvé Yves, Segura Francisco J, Martínez-Navarrete Gema, Tamarit José Manuel, Fuentes-Broto Lorena, Sanchez-Cano Ana, Pinilla Isabel
Department of Physiology, Genetics and Microbiology, University of Alicante Alicante, Spain.
Departments of Ophthalmology and Physiology, University of Alberta Edmonton, AB, Canada.
Front Neuroanat. 2014 Dec 22;8:151. doi: 10.3389/fnana.2014.00151. eCollection 2014.
The P23H rhodopsin mutation is an autosomal dominant cause of retinitis pigmentosa (RP). The degeneration can be tracked using different anatomical and functional methods. In our case, we evaluated the anatomical changes using Spectral-Domain Optical Coherence Tomography (SD-OCT) and correlated the findings with retinal thickness values determined by immunocytochemistry.
Pigmented rats heterozygous for the P23H mutation, with ages between P18 and P180 were studied. Function was assessed by means of optomotor testing and ERGs. Retinal thicknesses measurements, autofluorescence and fluorescein angiography were performed using Spectralis OCT. Retinas were studied by means of immunohistochemistry.
Between P30 and P180, visual acuity decreased from 0.500 to 0.182 cycles per degree (cyc/deg) and contrast sensitivity decreased from 54.56 to 2.98 for a spatial frequency of 0.089 cyc/deg. Only cone-driven b-wave responses reached developmental maturity. Flicker fusions were also comparable at P29 (42 Hz). Double flash-isolated rod-driven responses were already affected at P29. Photopic responses revealed deterioration after P29.A reduction in retinal thicknesses and morphological modifications were seen in OCT sections. Statistically significant differences were found in all evaluated thicknesses. Autofluorescence was seen in P23H rats as sparse dots. Immunocytochemistry showed a progressive decrease in the outer nuclear layer (ONL), and morphological changes. Although anatomical thickness measures were significantly lower than OCT values, there was a very strong correlation between the values measured by both techniques.
In pigmented P23H rats, a progressive deterioration occurs in both retinal function and anatomy. Anatomical changes can be effectively evaluated using SD-OCT and immunocytochemistry, with a good correlation between their values, thus making SD-OCT an important tool for research in retinal degeneration.
P23H视紫红质突变是视网膜色素变性（RP）的常染色体显性病因。可使用不同的解剖学和功能方法追踪这种变性。在我们的研究中，我们使用光谱域光学相干断层扫描（SD-OCT）评估解剖学变化，并将结果与通过免疫细胞化学测定的视网膜厚度值相关联。
研究了年龄在P18至P180之间的P23H突变杂合子有色大鼠。通过视动测试和视网膜电图评估功能。使用Spectralis OCT进行视网膜厚度测量、自发荧光和荧光素血管造影。通过免疫组织化学研究视网膜。
在P30至P180之间，视力从每度0.500周期降至0.182周期（周/度），对于0.089周/度的空间频率，对比敏感度从54.56降至2.98。只有视锥细胞驱动的b波反应达到发育成熟。在P29时闪烁融合也相当（42 Hz）。双闪光分离的视杆细胞驱动反应在P29时就已受到影响。明视觉反应在P29后显示恶化。在OCT切片中可见视网膜厚度减少和形态学改变。在所有评估的厚度中均发现统计学上的显著差异。在P23H大鼠中，自发荧光表现为稀疏的点状。免疫细胞化学显示外核层（ONL）逐渐减少以及形态学变化。尽管解剖学厚度测量值明显低于OCT值，但两种技术测量的值之间存在非常强的相关性。
在有色P23H大鼠中，视网膜功能和解剖结构均出现进行性恶化。使用SD-OCT和免疫细胞化学可以有效地评估解剖学变化，它们的值之间具有良好的相关性，因此使SD-OCT成为视网膜变性研究的重要工具。
