2026-03-13T05:45:39Z https://hama-med.repo.nii.ac.jp/oai

oai:hama-med.repo.nii.ac.jp:00000031 2024-02-20T07:49:58Z 1:11

Glycine cleavage system in neurogenic regions Ichinohe, Akiko Kure, Shigeo Mikawa, Sumiko Ueki, Takatoshi Kojima, Kanako Fujiwara, Kazuko Iinuma, Kazuie Matsubara, Yoichi Sato, Kohji open access glycine glycine cleavage system non-ketotic hyperglycinemia rat neural stem cell 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 non-ketotic 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 non-ketotic hyperglycinemia Blackwell Publishing 2004-03 eng journal article AM http://hdl.handle.net/10271/46 https://hama-med.repo.nii.ac.jp/records/31 15128390 https://doi.org/10.1111/j.0953-816X.2004.03345.x 0953-816X 1460-9568 European Journal of Neuroscience 19 9 2365 2370 https://hama-med.repo.nii.ac.jp/record/31/files/EJN-19_2004.pdf application/pdf 675.7 kB 2018-08-27