Variability in rate of cone degeneration in the retinal degeneration (rd/rd) mouse

Variability in rate of cone degeneration in the retinal degeneration (rd/rd) mouse
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DOI:
10.1006/exer.1997.0308
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发表时间:
1997-07-01
影响因子:
3.4
通讯作者:
Steinberg, RH
Steinberg, RH
中科院分区:
医学3区
文献类型:
--
作者:
LaVail, MM;Matthes, MT;Steinberg, RH

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对患有遗传性视网膜变性的rd/rd小鼠的视网膜在出生后60-66天进行组织学检查,在这个年龄,大多数视杆细胞已经退化并消失,但仍然存在大量视锥细胞。我们观察到出乎意料的半球不对称性和幸存视锥细胞数量的巨大变化。在大多数视网膜中,下半球中存活的视锥细胞明显多于上半球,尽管在大约 15% 的动物中,半球不对称性不存在或被逆转。不同动物的存活视锥细胞数量差异很大。在上半球范围内,范围为 3-30,即 10 倍;在下半球范围内,范围为 7-51,即大于 7 的因子。如果忽略特定的半球,数量范围为 3-51,系数为 17。这些发现对于检查这种广泛研究的突变体退化后期的视锥细胞存活具有重要意义,包括使用移植、基因治疗或存活因子的治疗研究,以及使用视锥细胞特异性标记物鉴定存活细胞。 (C) 1997 学术出版社有限公司。
The retinas of rd/rd mice with inherited retinal degeneration were examined histologically at postnatal days 60-66, an age when most rod cells already have degenerated and disappeared but when a significant number of cones are still present. We observed an unexpected hemispheric asymmetry and large variability in the number of surviving cones. Significantly more cones survived in the inferior than in the superior hemisphere in most retinas, although in about 15% of animals the hemispheric asymmetry was absent or was reversed. The number of surviving cones was highly variable from animal to animal. ranging from 3-30, a factor of 10, within the superior hemisphere, and from 7-51, a factor greater than 7, in the inferior hemisphere. If the specific hemisphere was ignored, the number ranged from 3-51, a factor of 17. These findings have significance for the examination of cone survival in the late stages of degeneration in this widely studied mutant, including therapeutic studies using transplantation, gene therapy or survival factors, as well as for the identification of surviving cells using cone-specific markers. (C) 1997 Academic Press Limited.