胶原及细胞外基质与先天性玻璃体异常  

Extracellular matrix and associated congenital vitreous abnormalities

作　　者：黄厚斌[1,2,3] Huang Houbin(Department of Ophthalmology,Hainan Hospital of PLA General Hospital,Sanya 572013,China;Senior Department of Ophthalmology,PLA General Hospital,Beijing 100853,China;The Second School of Clinical Medicine,Southern Medical University,Guangzhou 510280,China)

机构地区：[1]解放军总医院海南医院眼科,三亚572013 [2]解放军总医院眼科医学部,北京100853 [3]南方医科大学第二临床医学院,广州510280

出　　处：《眼科》2023年第2期89-97,共9页Ophthalmology in China

基　　金：海南省卫生健康行业科研项目(22A200351);军委后勤保障部保健专项科研课题面上项目(19BJZ39);军队后勤科研项目保健专项课题(23BJZ37)。

摘　　要：玻璃体是一种细胞外基质,主要成分包括胶原、透明质酸和蛋白聚糖等,编码其蛋白质成分的基因突变时,可导致不同表现的先天性玻璃体异常,如膜状、串珠状、空腔状,以及近视、视网膜脱离等。全面认识与玻璃体相关的细胞外基质成分,有助于深刻了解基因异常导致的先天性玻璃体视网膜疾病。Vitreous is a kind of extracellular matrix in nature,consisting of collagen,hyaluronan,proteoglycan,etc.Mutations of the genes coding the protein components would result in various congenital vitreous abnormalities including membrane,beaded or hypoplastic phenotype.High myopia and retinal detachment are commonly associated.Comprehensively knowing the extracellular matrix components composing the vitreous is really helpful to understand deeply the congenital inherited vitreoretinopathies.

关 键 词：玻璃体 细胞外基质 Stickler综合征 Knobloch综合征 Wagner综合征 MARFAN综合征 

分 类 号：R776.4[医药卫生—眼科]