Glycine cleavage system in neurogenic regions
Glycine cleavage system in neurogenic regions
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DOI：
10.1111/j.0953-816x.2004.03345.x
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发表时间：
2004-05-01
影响因子：
3.4
通讯作者：
Sato, K
中科院分区：
文献类型：
--
作者：
Ichinohe, A;Kure, S;Sato, K
甘氨酸裂解系统(GCS)是降解甘氨酸和为DNA合成提供5，10-亚甲基四氢叶酸的重要酶复合体。这一系统的遗传性缺陷导致非酮症高血糖，其特征是严重的神经系统症状和频繁的脑畸形。虽然高水平的甘氨酸被认为是导致上述问题的原因，但该病的详细发病机制仍不清楚。在这里，我们发现GCS在大鼠胚胎神经干/祖细胞的神经上皮细胞中大量表达，并且这种表达被传递到放射状胶质-星形胶质细胞谱系，并且在出生后的神经发生区有显著的表达。提示GCS在神经发生中起重要作用，提示GCS缺陷引起的神经发生障碍可能是非酮症高血糖的主要发病机制。
The glycine cleavage system (GCS) is the essential enzyme complex for degrading glycine and supplying 5,10-methylenetetrahydrofolate for DNA synthesis. Inherited deficiency of this system causes nonketotic hyperglycinemia, characterized by severe neurological symptoms and frequent association of brain malformations. Although high levels of glycine have been considered to cause the above-mentioned problems, the detailed pathogenesis of this disease is still unknown. Here we show that GCS is abundantly expressed in rat embryonic neural stem/progenitor cells in the neuroepithelium, and this expression is transmitted to the radial glia-astrocyte lineage, with prominence in postnatal neurogenic regions. These data indicate that GCS plays important roles in neurogenesis, and suggest that disturbance of neurogenesis induced by deficiency of GCS may be the main pathogenesis of nonketotic hyperglycinemia.