Bone abnormalities and unilateral eye deterioration in aspartate-glutamate carrier deficient mice

• Project/Area Number: 19500365
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Single-year Grants
• Section: 一般
• Research Field: Laboratory animal science
• Research Institution: Kagoshima University
• Principal Investigator: IIJIMA Mikio Kagoshima University, 大学院・医歯学総合研究科, 助教 (00305111)
• Co-Investigator(Kenkyū-buntansha): NAKAGAWA Shirou 鹿児島大学, 大学院・医歯学総合研究科, 教授 (70073666)
• Project Period (FY): 2007 – 2009
• Project Status: Completed (Fiscal Year 2009)
• Budget Amount: ¥4,420,000 (Direct Cost: ¥3,400,000、Indirect Cost: ¥1,020,000); Fiscal Year 2009: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000); Fiscal Year 2008: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000); Fiscal Year 2007: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
• Keywords: アスパラギン酸グルタミン酸輸送体 / アスパラギン酸・グルタミン酸輸送体 / 脳・神経 / 骨

Research Abstract

Aspartate-glutamate carrier deficient mice (citrin^<-/->, aralar^<+/-> mice) demonstrated growth retarded rate and bone abnomalities compared with their citrin-KO (citrin^<-/->, aralar^<+/+> mice) littermates. Citrin^<-/->, aralar^<+/-> mice fail to display hyperammonemia after oral administration of sucrose which is one of features of human type II citrullinemia model mice, citrin^<-/->, mGPDH^<-/-> mice. No citrin^<-/->, aralar^<+/-> mice which is suffer from unilateral eye deterioration demonstrated both eye aberrations up to 1 year.

Research Products

• [Journal Article] Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) as a cause of liver disease in infants in the UK. (2009)
  • Author(s): Hutchin T, Iijima M, (他10名、8番目)
  • Journal Title: J Inherit. Metab. Dis.
  • Peer Reviewed
• [Journal Article] Neonatal intrahepatic cholestasis caused by citrin deficiency(NICCD)as a cause of liver disease in infants in the UK (2009)
  • Author(s): Hutchin T
  • Journal Title: J Inherit Metab Dis
  • Peer Reviewed
• [Journal Article] Citrin/mitochondrial glycerol-3-phosphate dehydrogenase double knock-out mice recapitulate features of human citrin deficiency. (2007)
  • Author(s): Saheki T, Iijima M, (他6名、2番目)
  • Journal Title: J. Biol. Chem. 282 Pages: 25041-25052
  • Peer Reviewed
• [Journal Article] Ca2+ activation kinetics of the two aspartate-glutamate mitochondrial carriers aralar and citrin: Role in heart malate-aspartate NADH shuttle. (2007)
  • Author(s): Contreras L, Gomez-Peurtas P, Iijima M, Kobayashi K, Saheki T, Satrustegui J
  • Journal Title: J. Biol. Chem. 282 Pages: 7098-7196
  • Peer Reviewed
• [Journal Article] Ca^<2+> activation kinetics of the two aspartate-glutamate mitochondrial carriers aralar and citrin: Role in heart malate-aspartate NADH shuttle. (2007)
  • Author(s): Contreras L, Gomez-Peurtas P, Iijima M, Kobayashi K, Saheki T, Satrustegui J
  • Journal Title: J. Biol. Chem. 282 Pages: 7098-7106
  • Peer Reviewed
• [Journal Article] Citrin/mitochondrial glycerol-3-phosphate dehydrogenase double knock-out mice recapitulate features of human citrin deficiency. (2007)
  • Author(s): Saheki T, Iijima M, Li MX, Kobayashi K, Horiuchi M, Ushikai M, Okumura F, Meng XJ, Inoue I, Tajima A, Moriyama M, Eto K, Kadowaki T, Sinasac DS, Tsui LC, Tsuji M, Okano A, Kobayashi T
  • Journal Title: J. Biol. Chem. 282 Pages: 25041-25052
  • Peer Reviewed
• [Presentation] Citrin/mGPDHダブルKOマウスを用いるシトリン欠損症の治療法の開発 (2008)
  • Author(s): 佐伯武頼, 小林圭子, 飯島幹雄
  • Organizer: 第50回日本先天代謝異常学会総会・第7回アジア先天代謝異常学会
  • Place of Presentation: 米子
• [Presentation] Citrin/mGPDHダブルKOマウスを用いるシトリン欠損症の治療法の開発 (2008)
  • Author(s): 佐伯武頼, 小林圭子, 飯島幹雄.
  • Organizer: 第50回日本先天代謝異常学会総会・第7回アジア先天代謝異常学会
  • Place of Presentation: 米子コンベンションセンター(米子)
• [Remarks]
  • URL: http://www.kufm.kagoshima-u.ac.jp/~biocheml/
• [Remarks]
  • URL: http://www.kufm.kagoshima-u.ac.jp/~biocheml/