Structural Biophysics Group, School of Optometry and Vision Sciences, Cardiff University, Cardiff, UK.
Biophys J. 2013 Jun 18;104(12):2586-94. doi: 10.1016/j.bpj.2013.04.053.
Avian vision diseases in which eye growth is compromised are helping to define what governs corneal shape and ultrastructural organization. The highly specific collagen architecture of the main corneal layer, the stroma, is believed to be important for the maintenance of corneal curvature and hence visual quality. Blindness enlarged globe (beg) is a recessively inherited condition of chickens characterized by retinal dystrophy and blindness at hatch, with secondary globe enlargement and loss of corneal curvature by 3-4 months. Here we define corneal ultrastructural changes as the beg eye develops posthatch, using wide-angle x-ray scattering to map collagen fibril orientation across affected corneas at three posthatch time points. The results disclosed alterations in the bulk alignment of corneal collagen in beg chicks compared with age-matched controls. These changes accompanied the eye globe enlargement and corneal flattening observed in affected birds, and were manifested as a progressive loss of circumferential collagen alignment in the peripheral cornea and limbus in birds older than 1 month. Progressive remodeling of peripheral stromal collagen in beg birds posthatch may relate to the morphometric changes exhibited by the disease, likely as an extension of myopia-like scleral remodeling triggered by deprivation of a retinal image.
在影响眼睛生长的禽类视觉疾病的研究中,人们逐渐认识到哪些因素在控制角膜的形状和超微结构组织。角膜基质层的主要胶原结构高度特异,人们认为这对于维持角膜曲率和视觉质量很重要。盲目性眼球增大症(beg)是一种常染色体隐性遗传疾病,雏鸡表现为视网膜营养不良和孵化时失明,3-4 月龄时出现眼球增大和角膜曲率丧失。本研究通过广角 X 射线散射技术,在三个出生后时间点检测受影响角膜的胶原纤维取向,来定义 beg 眼出生后的角膜超微结构变化。结果显示,与同龄对照相比,beg 雏鸡的角膜胶原整体排列发生了改变。这些变化伴随着眼球增大和角膜变平,在 1 月龄以上的患病鸟类中表现为周边角膜和角膜缘的环形胶原排列逐渐丧失。出生后 beg 雏鸡的周边基质胶原进行性重塑可能与疾病表现出的形态变化有关,这可能是由于剥夺视网膜图像后,类似近视的巩膜重塑的延伸。