title: Glycine cleavage system in neurogenic regions.
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 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.
authors.affiliation: Department of Medical Genetics, Tohoku University School of Medicine, Sendai 980-8574, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Ichinohe
authors.forename: Shigeo
authors.initials: S
authors.lastname: Kure
authors.forename: Sumiko
authors.initials: S
authors.lastname: Mikawa
authors.forename: Takatoshi
authors.initials: T
authors.lastname: Ueki
authors.forename: Kanako
authors.initials: K
authors.lastname: Kojima
authors.forename: Kazuko
authors.initials: K
authors.lastname: Fujiwara
authors.forename: Kazuie
authors.initials: K
authors.lastname: Iinuma
authors.forename: Yoichi
authors.initials: Y
authors.lastname: Matsubara
authors.forename: Kohji
authors.initials: K
authors.lastname: Sato
mesh_terms: D000594:Amino Acid Oxidoreductases / Q000235:genetics / Q000378:metabolism*; D027322:Amino Acid Transport System X-AG / Q000235:genetics / Q000378:metabolism; D000818:Animals; D000831:Animals, Newborn; D001921:Brain / Q000166:cytology / Q000502:physiology*; D001923:Brain Chemistry; D002352:Carrier Proteins / Q000235:genetics / Q000378:metabolism*; D002478:Cells, Cultured; D004622:Embryo, Mammalian; D004847:Epithelial Cells / Q000166:cytology; D018507:Gene Expression Regulation, Developmental / Q000502:physiology*; D007150:Immunohistochemistry / Q000379:methods; D008099:Liver / Q000201:enzymology; D009097:Multienzyme Complexes / Q000235:genetics / Q000378:metabolism*; D009457:Neuroglia / Q000201:enzymology*; D009474:Neurons / Q000201:enzymology*; D012333:RNA, Messenger / Q000096:biosynthesis; D051381:Rats; D017208:Rats, Wistar; D020133:Reverse Transcriptase Polymerase Chain Reaction / Q000379:methods; D014166:Transferases / Q000235:genetics / Q000378:metabolism*
chemical_list: D027322:Amino Acid Transport System X-AG; D002352:Carrier Proteins; D009097:Multienzyme Complexes; D012333:RNA, Messenger; C064525:glycine cleavage system; D000594:Amino Acid Oxidoreductases; D014166:Transferases