Lee Jiwoon, Gross Jeffrey M
Section of Molecular Cell and Developmental Biology, The University of Texas at Austin, Austin, Texas 78712, USA.
Invest Ophthalmol Vis Sci. 2007 Jun;48(6):2483-90. doi: 10.1167/iovs.06-1211.
In this study recessive zebrafish mutations in the genes encoding laminin beta1 (lamb1) and laminin gamma1 (lamc1) were used to determine the functions of these laminin proteins during ocular basement membrane formation and during zebrafish eye development.
Ocular defects in lamb1 and lamc1 mutants were characterized by using a combination of histology, immunohistochemistry, in situ hybridization, and transmission electron microscopy.
The results demonstrated that zebrafish lamb1 and lamc1 mutants possess defects in two ocular basement membranes--the lens capsule and the inner limiting membrane--whereas Bruch's membrane is largely unaffected. lamb1 and lamc1 mutants possess severe lens dysplasias that result from a compromise in lens capsule integrity. Inner limiting membrane continuity is irregular in these mutants, and these irregularities result in small retinal ectopias that extend from the retina into the interstitial space between the retina and the lens. At late embryonic stages (e.g., 5-7 days after fertilization), retinal lamination defects are also observed in a subset of laminin mutants.
The results demonstrate that laminin beta1 and -gamma1 containing laminins are essential for the integrity of the lens capsule basement membrane and inner limiting membrane in the zebrafish eye.
在本研究中，利用编码层粘连蛋白β1（lamb1）和层粘连蛋白γ1（lamc1）的基因中的隐性斑马鱼突变，来确定这些层粘连蛋白在眼基底膜形成和斑马鱼眼睛发育过程中的功能。
通过组织学、免疫组织化学、原位杂交和透射电子显微镜等方法相结合，对lamb1和lamc1突变体的眼部缺陷进行表征。
结果表明，斑马鱼lamb1和lamc1突变体在两个眼基底膜——晶状体囊膜和内界膜——存在缺陷，而布鲁赫膜基本未受影响。lamb1和lamc1突变体存在严重的晶状体发育异常，这是由于晶状体囊膜完整性受损所致。这些突变体的内界膜连续性不规则，这些不规则导致小的视网膜异位，从视网膜延伸到视网膜与晶状体之间的间隙。在胚胎后期阶段（例如，受精后5 - 7天），在一部分层粘连蛋白突变体中也观察到视网膜分层缺陷。
结果表明，含有层粘连蛋白β1和γ1的层粘连蛋白对于斑马鱼眼睛中晶状体囊膜基底膜和内界膜的完整性至关重要。
