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青光眼白化鹌鹑的视觉系统退化

Visual system degeneration in the glaucomatous albino quail.

作者信息

Weidner C, Repérant J, Miceli D, Rio J P, Desroches A M, Kirpitchnikova E

机构信息

Institut des Neurosciences du CNRS, Université Paris VI, France.

出版信息

J Hirnforsch. 1988;29(3):299-314.

PMID:3418116
Abstract

The peripheral (eye, retina, optic nerve) and central (primary optic tracti and centers, centrifugal visual tractus and nucleus) visual system of an imperfect albino quail mutant with a sex linked recessive gene was examined in 32 specimens ages 1 week - 16 months-hatch using various histological techniques. During the first weeks the visual system was normal and comparable in its overall organization to that found in the pigmented quail. However, the ipsilateral retinal projections were observed to be weaker in the young mutant, then completely disappeared two months after birth. Initial signs of the bupthalmos, a form of spontaneous glaucoma, appeared between the 3rd and 5th months. This was characterized by a distention of the eye linked to an increase in intraocular pressure. The pathological process was progressive and at 16 months the eye was very prominent, the anterior chamber deep and a large and globular cornea was noted. The glaucoma progressively induced different histopathological changes in the visual system including: cupping of the optic disc, degeneration of optic axons and their parent ganglion and centrifugal cells and cavernous degeneration. All of these phenomena were identifiable at about the 10th post-natal month and progressed in a relatively constant and orderly manner. The retinal projections to the nucleus ectomamillaris, ventral and lateral optic tectum and ventral pretectum were the first to degenerate. The degeneration of optic fibers attaining the dorsal pretectum and dorsal thalamus occurred later. Furthermore the retrograde degeneration in the centrifugal isthmo-optic nucleus progressed from the external to the internal pole. The mechanisms involved in the selective degeneration of centrifugal and centripetal optic fibers is discussed.

摘要

利用各种组织学技术,对32只年龄在1周龄至16个月龄(孵化后)的携带性连锁隐性基因的不完全白化鹌鹑突变体的外周视觉系统(眼睛、视网膜、视神经)和中枢视觉系统(初级视束及其中枢、离心视觉束及其核)进行了检查。在最初几周,视觉系统是正常的,其整体组织结构与有色鹌鹑的视觉系统相当。然而,在幼年突变体中观察到同侧视网膜投射较弱,然后在出生后两个月完全消失。眼球突出(一种自发性青光眼的形式)的最初迹象出现在第3至5个月之间。其特征是眼睛扩张与眼内压升高有关。病理过程是渐进性的,在16个月时,眼睛非常突出,前房很深,角膜大且呈球形。青光眼逐渐在视觉系统中引发不同的组织病理学变化,包括:视盘凹陷、视神经轴突及其母神经节和离心细胞的退化以及海绵状变性。所有这些现象在出生后约第10个月时均可识别,并以相对恒定和有序的方式发展。向乳头外侧核、腹侧和外侧视顶盖以及腹侧顶盖前区的视网膜投射是最先退化的。到达背侧顶盖前区和背侧丘脑的视神经纤维的退化发生得较晚。此外,离心性峡视核的逆行性退化从外极向内极发展。文中讨论了离心性和向心性视神经纤维选择性退化所涉及的机制。

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