Establishment of primate model for progeroid syndromes derived from sporadic patient Japanese monkeys
Project/Area Number |
15K07235
|
Research Category |
Grant-in-Aid for Scientific Research (C)
|
Allocation Type | Multi-year Fund |
Section | 一般 |
Research Field |
Physical anthropology
|
Research Institution | Kyoto University |
Principal Investigator |
Oishi Takao 京都大学, 霊長類研究所, 准教授 (40346036)
|
Research Collaborator |
HIRAI Hirohisa
IMAMURA Masanori
GO Yasuhiro
DONG Cho
|
Project Period (FY) |
2015-04-01 – 2018-03-31
|
Project Status |
Completed (Fiscal Year 2017)
|
Budget Amount *help |
¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2017: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2016: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2015: ¥2,470,000 (Direct Cost: ¥1,900,000、Indirect Cost: ¥570,000)
|
Keywords | 早老症 / モデル動物 / ニホンザル / ムコ多糖症 / リソソーム病 / 自然発症 / 動物モデル / 難病 |
Outline of Final Research Achievements |
To identify the cause of progeroid symptoms of a Japanese monkey N416 (PLos One, 2014), element contents were measured in various organs. Amount of zinc in the heart, the liver, the kidney, but not in the lung or the cerebral cortex, or the hippocampus of N416, were lower than in that of normal monkeys. As zinc is essential for functions of DNA repair enzymes or antioxidant enzymes, low contents of zinc in these organs may lead to premature aging symptoms, though it does not account for shrinkage of the brain. Three Japanese monkeys, Mff2389 and two sisters, show gargoyle face. X-ray analysis and urine test supported that they suffer from mucopolysaccharidosis (MPS). Exosome analysis suggested that a homozygous missense mutation in IDUA gene lead to the symptoms in Mff2389. Genotype test showed that the sisters of Mff2389 were homozygous and half of the non-pedigree were heterozygous. This group can be a good source for the primate model for MPS, type I.
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Report
(4 results)
Research Products
(10 results)