Targeted ablation of NrCAM or ankyrin-B results in disorganized lens fibers leading to cataract formation.
Targeted ablation of NrCAM or ankyrin-B results in disorganized lens fibers leading to cataract formation.
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DOI：
10.1083/jcb.200104038
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发表时间：
2001-07-09
影响因子：
7.8
通讯作者：
Rathjen, F G
中科院分区：
文献类型：
--
作者：
More, M I;Kirsch, F P;Rathjen, F G
NgCAM相关细胞粘附分子（NrCAM）是L1亚群的免疫球蛋白超家族成员，其在细胞内与锚蛋白相互作用。我们发现，NrCAM的情况下，在小鼠中的成熟白内障的形成的原因，而在脊髓中线或本体感受轴突侧支连合轴突的显着寻路错误没有检测到。白内障是视力损害的最常见原因，在NrCAM缺陷小鼠中通过透镜纤维的解体，随后细胞崩解和细胞碎片的积累而产生。在胚胎发育后期，纤维细胞的解体在组织学上变得不同，包括细胞骨架和含有连接蛋白50的间隙连接的异常。此外，对锚蛋白-B突变小鼠晶状体的分析也揭示了出生后第1天透镜纤维的紊乱，与缺乏NrCAM所产生的紊乱难以区分，表明需要NrCAM和锚蛋白-B来维持透镜纤维细胞之间的接触。此外，这些研究还提供了NrCAM和锚蛋白-B之间相互作用的遗传证据。
The NgCAM-related cell adhesion molecule (NrCAM) is an immunoglobulin superfamily member of the L1 subgroup that interacts intracellularly with ankyrins. We reveal that the absence of NrCAM causes the formation of mature cataracts in the mouse, whereas significant pathfinding errors of commissural axons at the midline of the spinal cord or of proprioceptive axon collaterals are not detected. Cataracts, the most common cause of visual impairment, are generated in NrCAM-deficient mice by a disorganization of lens fibers, followed by cellular disintegration and accumulation of cellular debris. The disorganization of fiber cells becomes histologically distinct during late embryonic development and includes abnormalities of the cytoskeleton and of connexin50-containing gap junctions. Furthermore, analysis of lenses of ankyrin-B mutant mice also reveals a disorganization of lens fibers at postnatal day 1, indistinguishable from that generated by the absence of NrCAM, indicating that NrCAM and ankyrin-B are required to maintain contact between lens fiber cells. Also, these studies provide genetic evidence of an interaction between NrCAM and ankyrin-B.