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

天冬氨酸-谷氨酸载体缺陷小鼠的骨骼异常和单侧眼睛退化

• 批准号: 19500365
• 负责人: IIJIMA Mikio
• 金额: $ 2.83万
• 依托单位: Kagoshima University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2007
• 资助国家: 日本
• 起止时间: 2007 至 2009
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-19500365/
• 关键词: 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

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.

天冬氨酸-谷氨酸载体缺陷小鼠（citrin^<-/->，arrhythmia ^<+/->小鼠）与其citrin-KO小鼠（citrin^<-/->，arrhythmia ^<+/+>小鼠）同窝仔相比表现出生长迟缓率和骨异常。在口服给药蔗糖后，Citrin^<-/->、mGPDH ^<+/->小鼠未表现出高氨血症，这是人II型瓜氨酸血症模型小鼠Citrin^<-/->、mGPDH^<-/->小鼠的特征之一。患有单眼退化的柠檬酸A <-/->、arrhythmia <+/->小鼠在长达1年的时间内均未表现出双眼像差。

项目成果

Citrin/Mitochondrial glycerol-3-phosphate dehydrogenase double knock-out mice recapitulate features of human citrin deficiency

• DOI: 10.1074/jbc.m702031200
• 发表时间: 2007-08-24
• 期刊: JOURNAL OF BIOLOGICAL CHEMISTRY
• 影响因子: 4.8
• 作者: Saheki, Takeyori;Iijima, Mikio;Kobayashi, Tsuyoshi
• 通讯作者: Kobayashi, Tsuyoshi