CAJ | 학술논문

目的比较野生型和K141N突变型小分子热休克蛋白22( HSP22)在原代培养小鼠皮质神经元轴突中的转运情况.方法将构建好的含有人类野生型和K141N突变型HSP22基因的质粒(pCAGGS-HA-wtHSP22和pCAGGS-HA-K141N HSP22)分别与pEGFP-N1共转染小鼠皮质神经元,转染后观察野生型和K141N突变型HSP22蛋白在小鼠皮质神经元轴突内的转运情况,分析野生型和K141N突变型HSP22蛋白在轴突内的转运距离.结果野生型HSP22蛋白在皮质神经元内被转运到轴突的最远端[转运距离(637±117)μm],K141N突变型HSP22蛋白仅被转运到轴突的近端[转运距离(29±12)μm],野生型和K141N突变型HSP22蛋白在轴突中被转运的距离差异具有统计学意义.结论 K141N突变型HSP22蛋白在轴突内的转运障碍可能在CMT2L型的发病中起着重要作用.
목적 비교야생형화K141N돌변형소분자열휴극단백22( HSP22)재원대배양소서피질신경원축돌중적전운정황.방법 장구건호적함유인류야생형화K141N돌변형HSP22기인적질립(pCAGGS-HA-wtHSP22화pCAGGS-HA-K141N HSP22)분별여pEGFP-N1공전염소서피질신경원,전염후관찰야생형화K141N돌변형HSP22단백재소서피질신경원축돌내적전운정황,분석야생형화K141N돌변형HSP22단백재축돌내적전운거리.결과 야생형HSP22단백재피질신경원내피전운도축돌적최원단[전운거리(637±117)μm],K141N돌변형HSP22단백부피전운도축돌적근단[전운거리(29±12)μm],야생형화K141N돌변형HSP22단백재축돌중피전운적거리차이구유통계학의의.결론 K141N돌변형HSP22단백재축돌내적전운장애가능재CMT2L형적발병중기착중요작용.
Objective To compare the axonal transport of wild-type (WT) and K141N mutant HSP22 in transfected primary cultured cortical neurons.Methods The plasmid (pCAGGS-HA-wtHSP22 or pCAGGS-HA-K141NHSP22) with WT or K141N mutant HSP22 gene and a GFP-expressing plasmid ( pEGFP-N1 ) were co-transfected respectively into primary cultured cortical neurons.The axonal transport of WT and K141N mutant HSP22 was observed.And the distance traveled by WT and K141N mutant HSP22 was analyzed.Results The WT HSP22 was transported within axons and uniformly present throughout the entire length of axons.K141N mutant HSP22 failed to be transported to the same extent and was present only in cell body and proximal portion of axons.Analysis of distance traveled revealed that WT HSP22 traveled significantly further than the K141N mutant HSP22.Conclusion The axonal transport of K141N mutant HSP22 may play an important role in the pathogenesis of CMT2L.