Apoptosis, retinitis pigmentosa, and degeneration.

Apoptosis, retinitis pigmentosa, and degeneration.
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细胞凋亡、色素性视网膜炎和变性。

DOI:
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发表时间:
1994
期刊:
Biochemistry and cell biology = Biochimie et biologie cellulaire
影响因子:
--
通讯作者:
P. Wong
P. Wong
中科院分区:
--
文献类型:
--
作者:
P. Wong

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不同遗传性视网膜变性中感光细胞死亡的机制还不完全清楚。许多不同基因(如视紫红质、cGMP磷酸二酯酶的β亚基和外周蛋白)的突变已被确定为不同形式的人类色素性视网膜炎的主要遗传病变,色素性视网膜炎是遗传性失明的最常见原因之一。在所有情况下,无论具体的原发性遗传病变的障碍的表现是相似的,导致感光细胞变性和失明。最近的一个假设是,活性感光细胞死亡,这是这些遗传上不同的疾病的特征,是由一个共同的诱导细胞凋亡介导的。在本综述中,目前的证据,在视网膜色素变性和视网膜变性的几种不同的啮齿动物模型的视网膜细胞死亡过程中的主动细胞死亡进行了检查。
The mechanism of photoreceptor cell death in different inherited retinal degenerations is not fully understood. Mutations in a number of different genes (such as rhodopsin, the beta subunit of cGMP phosphodiesterase, and peripherin) have been identified as the primary genetic lesion in different forms of human retinitis pigmentosa, one of the most common causes of inherited blindness. In all cases the manifestation of the disorder regardless of the specific primary genetic lesion is similar, resulting in photoreceptor cell degeneration and blindness. A recent hypothesis is that the active photoreceptor cell death, which is characteristic of these genetically distinct disorders, is mediated by a common induction of apoptosis. In the present review, the current evidence for active cell death during retinal cell death in several different rodent models of retinitis pigmentosa and retinal degeneration is examined.
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