Identification and characterization of rod-derived cone viability factor
Identification and characterization of rod-derived cone viability factor
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DOI：
10.1038/ng1386
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发表时间：
2004-07-01
期刊：
影响因子：
30.8
通讯作者：
Sahel, JA
中科院分区：
文献类型：
--
作者：
Léveillard, T;Mohand-Saïd, S;Sahel, JA
视网膜色素变性是一种无法治疗的遗传性视网膜疾病，会导致失明。该病始于视杆感光细胞变性导致的夜间视力丧失，随后是视锥细胞感光细胞不可逆转的进行性丧失(1-3)。视锥细胞丢失是导致视力障碍的主要原因，因为视锥细胞对于白天和高视力是必不可少的(4)。它们的丢失是间接的，因为大多数与视网膜色素变性相关的基因都不在这些细胞中表达。我们之前已经证明，视杆分泌的因子对于视锥细胞的存活是必不可少的(5-8)。在这里，我们通过表达克隆的方法鉴定了一个这样的营养因子，并将其命名为杆源性锥体生存因子(RdCVF)。RdCVF是一种截短的硫氧还蛋白样蛋白，由光感受器特异表达。该蛋白的鉴定为视网膜色素变性的治疗提供了新的可能性。
Retinitis pigmentosa is an untreatable, inherited retinal disease that leads to blindness. The disease initiates with the loss of night vision due to rod photoreceptor degeneration, followed by irreversible, progressive loss of cone photoreceptor(1-3). Cone loss is responsible for the main visual handicap, as cones are essential for day and high-acuity vision(4). Their loss is indirect, as most genes associated with retinitis pigmentosa are not expressed by these cells. We previously showed that factors secreted from rods are essential for cone viability(5-8). Here we identified one such trophic factor by expression cloning and named it rod-derived cone viability factor (RdCVF). RdCVF is a truncated thioredoxin-like protein specifically expressed by photoreceptors. The identification of this protein offers new treatment possibilities for retinitis pigmentosa.