Glycine cleavage system in neurogenic regions

• Ichinohe, Akiko

• Kure, Shigeo

• Mikawa, Sumiko

• Ueki, Takatoshi

• Kojima, Kanako

• Fujiwara, Kazuko

• Iinuma, Kazuie

• Matsubara, Yoichi

• Sato, Kohji

Abstract

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

Journal

• European Journal of Neuroscience

  European Journal of Neuroscience 19 (9), 2365-2370, 2004-03

  Blackwell Publishing

Keywords

• glycine
• glycine cleavage system
• non-ketotic hyperglycinemia
• rat
• neural stem cell

Details

• CRID: 1050282676638540032
• NII Article ID: 120005312462
• ISSN: 14609568
• ISSN: 0953816X
• HANDLE: 10271/46
• Web Site: https://hama-med.repo.nii.ac.jp/records/31
• Text Lang: en
• Article Type: journal article
• Data Source:
  • IRDB
  • CiNii Articles