Wiggert B, van Veen T, Kutty G, Lee L, Nickerson J, Si J S, Nilsson S E, Chader G J, Narfström K
National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892.
Cell Tissue Res. 1994 Nov;278(2):291-8. doi: 10.1007/BF00414173.
Levels of interphotoreceptor retinoid-binding protein (IRBP) protein and message in retinas of Abyssinian cats homozygous for progressive rod-cone degeneration were determined at early ages, well before the onset of clinical retinal degeneration. IRBP gene expression was assessed by immunochemical quantitation of IRBP protein, and by Northern blotting and slot-blotting of total RNA using a human IRBP cDNA probe. Morphology was assessed by electron microscopy and immunocytochemistry. Levels of both IRBP protein and message in affected Abyssinian cat retinas were significantly reduced below normal as early as 4 weeks of age at the earliest stage of retinal disorientation. Opsin mRNA was more abundant in affected Abyssinian cat retinas than in control retinas. This was at least 1 year before the onset of clinical symptoms. The reduction in IRBP gene expression to levels significantly below normal well before the onset of retinal degeneration in affected Abyssinian cat retinas indicates that this represents a primary defect or at least an early problem that could itself cause adverse effects.
在患有进行性视杆-视锥细胞变性的纯合阿比西尼亚猫的视网膜中，早在临床视网膜变性出现之前的早期阶段，就对光感受器间类视黄醇结合蛋白（IRBP）的蛋白质和信使水平进行了测定。通过对IRBP蛋白进行免疫化学定量，以及使用人IRBP cDNA探针对总RNA进行Northern印迹和狭缝印迹来评估IRBP基因表达。通过电子显微镜和免疫细胞化学评估形态学。早在视网膜紊乱的最早阶段，即4周龄时，受影响的阿比西尼亚猫视网膜中的IRBP蛋白和信使水平就显著低于正常水平。视蛋白mRNA在受影响的阿比西尼亚猫视网膜中比在对照视网膜中更丰富。这至少发生在临床症状出现前1年。在受影响的阿比西尼亚猫视网膜中，早在视网膜变性出现之前，IRBP基因表达就降低到显著低于正常水平，这表明这代表了一个原发性缺陷，或者至少是一个本身可能会产生不利影响的早期问题。
