Long-term evaluation of Leber's hereditary optic neuropathy-like symptoms in rotenone administered rats.
Long-term evaluation of Leber's hereditary optic neuropathy-like symptoms in rotenone administered rats.
复制标题
鱼藤酮给药大鼠莱伯遗传性视神经病样症状的长期评估。
DOI：
10.1016/j.neulet.2014.12.004
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发表时间：
2015
影响因子：
2.5
通讯作者：
Thomas,BijuB
中科院分区：
文献类型：
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作者：
Zhang,Li;Liu,Laura;Philip,AnnL;Martinez,JuanC;Guttierez,JuanC;Marella,Mathieu;Patki,Gaurav;Matsuno-Yagi,Akemi;Yagi,Takao;Thomas,BijuB
Leber遗传性视神经病变（LHON）是一种影响视网膜神经节细胞（RGC）及其轴突的遗传性疾病，可导致中心视力丧失。本研究的目的是评价鱼藤酮微球进入大鼠上级丘（SC）给药后LHON症状。光学相干断层扫描（OCT）分析显示，在注射鱼藤酮的大鼠视网膜神经纤维层（RNFL）厚度的实质性损失。鱼藤酮治疗大鼠的视动测试显示头部跟踪反应下降。SC表面的电生理标测显示视觉诱发反应衰减;然而，在ERG数据中未观察到变化。鱼藤酮给药大鼠的疾病表现的进展模式与人类疾病症状有几个相似之处。这些具有LHON样症状的大鼠可以作为未来研究人员设计和实施可靠测试的模型，以评估LHON疾病治疗干预的有益效果。
Leber’s hereditary optic neuropathy (LHON) is an inherited disorder affecting the retinal ganglion cells (RGCs) and their axons that lead to the loss of central vision. This study is aimed at evaluating the LHON symptoms in rats administered with rotenone microspheres into the superior colliculus (SC). Optical coherence tomography (OCT) analysis showed substantial loss of retinal nerve fiber layer (RNFL) thickness in rotenone injected rats. Optokinetic testing in rotenone treated rats showed decrease in head-tracking response. Electrophysiological mapping of the SC surface demonstrated attenuation of visually evoked responses; however, no changes were observed in the ERG data. The progressive pattern of disease manifestation in rotenone administered rats demonstrated several similarities with human disease symptoms. These rats with LHON-like symptoms can serve as a model for future investigators to design and implement reliable tests to assess the beneficial effects of therapeutic interventions for LHON disease.