Photoreceptor cells as a source of fundus autofluorescence in recessive Stargardt disease.
Photoreceptor cells as a source of fundus autofluorescence in recessive Stargardt disease.
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DOI：
10.1002/jnr.24252
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发表时间：
2019-01
影响因子：
4.2
通讯作者：
Sparrow JR
中科院分区：
文献类型：
--
作者：
Paavo M;Lee W;Allikmets R;Tsang S;Sparrow JR
类双维甲酸荧光团是由维生素A醛的非酶反应形成的。隐性Stargardt病（STGD1）眼底斑点的短波自身荧光（SW-AF）提示与这些荧光团有关。通过多模态成像，我们试图阐明这种联系。在SW-AF图像中观察到的斑点通常与焦点共定位，显示近红外自身荧光信号减少或缺失，其来源是视网膜色素上皮（RPE）细胞中的黑色素。通过连续成像，在SW-AF图像中，NIR-AF的变化先于斑点高自身荧光的出现，并且NIR-AF图像中的斑点轮廓更大。SW-AF和NIR-AF图像中的斑点也对应于水平SD-OCT扫描中穿过光感受器可归因于波段的高反射病变。高反射性病变中断相邻OCT反射带，并伴有外核层变薄。这些SD-OCT结果可归因于感光细胞变性。在彩色编码的定量眼底自体荧光（qAF）图中，斑点的SW-AF强度明显增加和减少。在一些病例中，斑点从中央凹呈放射状扩散到偏心距约8°，超过这个范围，斑点以周向扩散为特征。由于NIR-AF信号来源于黑色素，这种自身荧光的丧失表明RPE萎缩，因此斑点的SW-AF不能用RPE中的类双维a脂褐素来解释。相反，我们认为类双维甲酸作为SW-AF信号的来源存在于光感受器中，而光感受器也是类双维甲酸合成的部位。隐性Stargardt病是一种黄斑变性的早期发病形式，其超自身荧光病灶通常是短波眼底自身荧光的特征。在颜色编码的定量自荧光图中，这些斑点的分布和强度变化是明显的。我们提供的证据表明，斑点是一组退化的感光细胞的表现。
Bisretinoid fluorophores form in photoreceptor outer segments from non-enzymatic reactions of vitamin A aldehyde. The short wavelength autofluorescence (SW-AF) of fundus flecks in recessive Stargardt disease (STGD1) suggests a connection to these fluorophores. Through multimodal imaging we sought to elucidate this link. Flecks observed in SW-AF images often colocalized with foci exhibiting reduced or absent near infrared autofluorescence signal, the source of which is melanin in retinal pigment epithelial (RPE) cells. With serial imaging, changes in NIR-AF preceded the onset of fleck hyperautofluorescence in SW-AF images and fleck profiles in NIR-AF images tended to be larger. Flecks in SW-AF and NIR-AF images also corresponded to hyperreflective lesions traversing photoreceptor-attributable bands in horizontal SD-OCT scans. The hyperreflective lesions interrupted adjacent OCT reflectivity bands and were associated with thinning of the outer nuclear layer. These SD-OCT findings are attributable to photoreceptor cell degeneration. Progressive increases and decreases in the SW-AF intensity of flecks were evident in color-coded quantitative fundus autofluorescence (qAF) maps. In some cases flecks appeared to spread radially from the fovea to approximately 8° of eccentricity, beyond which a circumferential spread characterized the distribution. Since the NIR-AF signal is derived from melanin and loss of this autofluorescence is indicative of RPE atrophy, the SW-AF of flecks cannot be accounted for by bisretinoid lipofuscin in RPE. Instead, we suggest that the bisretinoid serving as the source of the SW-AF signal, resides in photoreceptors, the cell that is also the site of bisretinoid synthesis. In recessive Stargardt disease, a early onset form of macular degeneration, hyperautofluorescent foci are often features of short wavelength fundus autofluorescence. In color-coded quantitative autofluorescence maps the distributions and intensity changes of these flecks are apparent. We provide evidence that flecks are a manifestation of groups of degenerating photoreceptor cells.
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