勒伯遗传性视神经病变中的光受体变化与m.G11778A突变
Qing-Mei Miao1, Yu-Fang Cheng1, Hong-Mei Zheng1
1Eye Center, Renmin Hospital of Wuhan University, Wuhan 430060, Hubei Province, China.
International journal of ophthalmology
|June 19, 2023
概括
勒伯遗传性视神经病变 (LHON) 在患者和无症状携带者中显著损害光受体功能. 在LHON中也观察到结构变化,特别是在外核层 (ONL) 中.
科学领域:
- 眼科医生 眼科 眼科
- 神经科学是一个神经科学.
- 遗传学 是一个遗传学.
背景情况:
- 勒伯遗传性视神经病变 (LHON) 是一种母体遗传的线粒体疾病.
- 它主要影响视神经,导致视力丧失.
- 了解光受体参与对于LHON管理至关重要.
研究的目的:
- 评估LHON患者和无症状携带者的功能和结构光受体变化.
- 使用全场电网红学 (FERG) 和光学连贯性断层扫描 (OCT) 进行评估.
主要方法:
- 横截面观察性研究,包括14名LHON患者,12名无症状携带者和14名对照.
- 在适应黑暗和光线条件下分析了FERG a波幅.
- 测量了OCT的外核层 (ONL),内/外段 (IS/OS) 和总光受体厚度.
主要成果:
- 在LHON患者和携带者中显著降低了FERGa波幅 (P<0.001).
- 与对照组相比,患者的ONL和总光受体层略有厚 (P<0.05).
- 与对照组相比,载体中的ONL和总光受体层较薄 (P<0.05).
结论:
- 在LHON患者和携带者中,光受体功能明显受损.
- 光受体形态的微妙变化,特别是ONL厚度存在.
- 这些发现凸显了LHON在视神经之外的广泛影响.
相关概念视频
Photoreceptors and Visual Pathways
6.1K
At the molecular level, visual signals trigger transformations in photopigment molecules, resulting in changes in the photoreceptor cell's membrane potential. The photon's energy level is denoted by its wavelength, with each specific wavelength of visible light associated with a distinct color. The spectral range of visible light, classified as electromagnetic radiation, spans from 380 to 720 nm. Electromagnetic radiation wavelengths exceeding 720 nm fall under the infrared category,...
6.1K
Anatomy of the Eyeball
7.2K
The eye is a spherical, hollow structure composed of three tissue layers. The outer layer — the fibrous tunic, comprises the sclera — a white structure — and the cornea, which is transparent. The sclera encompasses some of the ocular surface, most of which is not visible. However, the 'white of the eye' is distinctively visible in humans compared to other species. The cornea, a clear covering at the front of the eye, enables light penetration. The eye's middle...
7.2K


