Glycine cleavage system in neurogenic regions

Akiko Ichinohe, Shigeo Kure, Sumiko Mikawa, Takatoshi Ueki, Kanako Kojima, Kazuko Fujiwara, Kazuie Iinuma, Yoichi Matsubara, Kohji Sato

Research output: Contribution to journalArticlepeer-review

34 Citations (Scopus)


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 gliaastrocyte 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.

Original languageEnglish
Pages (from-to)2365-2370
Number of pages6
JournalEuropean Journal of Neuroscience
Issue number9
Publication statusPublished - 2004 May


  • Glycine
  • Glycine cleavage system
  • Neural stem cell
  • Nonketotic hyperglycinemia
  • Rat

ASJC Scopus subject areas

  • Neuroscience(all)


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