Abnormal development of hypoxanthine-guanine phosphoribosyltransferase-deficient CNS neuroblastoma
Abnormal development of hypoxanthine-guanine phosphoribosyltransferase-deficient CNS neuroblastoma
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DOI：
10.1016/s0006-8993(01)02909-2
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发表时间：
2001-11-09
期刊：
影响因子：
2.9
通讯作者：
Stacey, NC
中科院分区：
文献类型：
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作者：
Connolly, GP;Duley, JA;Stacey, NC
Lesch-Nyhan综合征包括一系列神经系统症状，由嘌呤回收酶、次黄嘌呤-鸟嘌呤磷酸核糖转移酶（HGPRT）缺乏引起。这种酶活性的缺乏如何影响神经系统的发育尚不清楚。在这项研究中，我们检测了N2aTG（一种hgprt缺乏的神经母细胞瘤及其hgprt阳性的对应物）在不同密度下增殖和分化的能力。综上所述，N2aTG细胞比N2a细胞增殖更少，分化更多，前者对低密度培养的影响更敏感。鉴于该神经母细胞瘤细胞系的同质性及其在神经元发育研究中的应用，本研究表明，N2aTG细胞可能被证明是研究Lesch-Nyhan综合征非多巴胺能神经元发育的合适体外模型。(C) 2001 Elsevier Science版权所有
Lesch-Nyhan syndrome encompasses a host of neurological symptoms, caused by a deficiency of the purine salvage enzyme, hypoxanthine-guanine phosphoribosyltransferase (HGPRT). How the absence of this enzymes activity affects development of the nervous system is unknown. In this study, we examined the ability of N2aTG, a HGPRT-deficient neuroblastoma and its HGPRT-positive counterpart to proliferate and differentiate at various densities. In summary, N2aTG cells proliferated less and differentiated more than N2a cells, with the former cells exhibiting enhanced sensitivity to the effects of low-density culture. Given the homogeneity of this neuroblastoma cell line and its use in studies of neuronal development, the present study indicates that N2aTG cells may prove a suitable in vitro model for the study of non-dopaminergic neuronal development in Lesch-Nyhan syndrome. (C) 2001 Elsevier Science BY All rights reserved.