Smith D S, Niethammer M, Ayala R, Zhou Y, Gambello M J, Wynshaw-Boris A, Tsai L H
Department of Pathology Harvard Medical School, 200 Longwood Avenue, Boston, Massachussetts 02115, USA.
Nat Cell Biol. 2000 Nov;2(11):767-75. doi: 10.1038/35041000.
Whereas total loss of Lis1 is lethal, disruption of one allele of the Lis1 gene results in brain abnormalities, indicating that developing neurons are particularly sensitive to a reduction in Lis1 dosage. Here we show that Lis1 is enriched in neurons relative to levels in other cell types, and that Lis1 interacts with the microtubule motor cytoplasmic dynein. Production of more Lis1 in non-neuronal cells increases retrograde movement of cytoplasmic dynein and leads to peripheral accumulation of microtubules. These changes may reflect neuron-like dynein behaviours induced by abundant Lis1. Lis1 deficiency produces the opposite phenotype. Our results indicate that abundance of Lis1 in neurons may stimulate specific dynein functions that function in neuronal migration and axon growth.
虽然Lis1的完全缺失是致命的,但Lis1基因的一个等位基因的破坏会导致大脑异常,这表明发育中的神经元对Lis1剂量的减少特别敏感。在这里,我们表明,相对于其他细胞类型的水平,Lis1在神经元中富集,并且Lis1与微管运动蛋白胞质动力蛋白相互作用。在非神经元细胞中产生更多的Lis1会增加胞质动力蛋白的逆行运动,并导致微管在周围积聚。这些变化可能反映了由丰富的Lis1诱导的类似神经元的动力蛋白行为。Lis1缺乏会产生相反的表型。我们的结果表明,神经元中Lis1的丰度可能会刺激在神经元迁移和轴突生长中起作用的特定动力蛋白功能。