2009 Fiscal Year Final Research Report
Analysis of Primary Immunodeficiency Syndromes using model mice.
| Project/Area Number |
20790731
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| Research Category |
Grant-in-Aid for Young Scientists (B)
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | Hiroshima University |
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Principal Investigator |
OKADA Satoshi Hiroshima University, 病院, 病院助教 (80457241)
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| Project Period (FY) |
2008 – 2009
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| Keywords | 免疫学 / 遺伝子 / 疾患モデルマウス / ELA2 |
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| Research Abstract |
We tried to generate Severe Congenital Neutropenia (SCN) model mouse. We produced wild-type (WT), P110L, R101Q and C194X ELA2 by PCR-based mutagenesis and introduced them into retrovirus Tet-on gene expression vector. We performed gene expression experiment by using HEK293 cells. Although ELA2 expression was observed, we could not analyze them because the ELA2 introduced cells result in cell death. Now, we are trying to control ELA2 expression level by control the concentration of doxycycline. After that, we are planning generate SCN model mouse by BM reconstitution using CD34 positive cells from human cord blood.
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Research Products
(20 results)
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[Journal Article] Clinical characteristics of perinatal lethal hypophosphatasia: A report of 6 cases2010
Author(s)
Nakamura-Utsunomiya A, Okada S, Hara K, Miyagawa S, Takeda K, Fukuhara R, Nakata Y, Hayashidani M, Tachikawa K, Michigami T, Ozono K, Kobayashi M
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Journal Title
Clinical Pediatric Endocrinology (in press)
Peer Reviewed
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[Journal Article] Neurodevelopmental abnormalities associated with severe congenital neutropenia due to the R86X mutation in the HAX1 gene2008
Author(s)
Ishikawa N, Okada S (equally contributed), Miki M, Shirao K, Kihara H, Tsumura M, Nakamura K, Kawaguchi H, Ohtsubo M, Yasunaga S, Matsubara K, Sako M, Hara J, Shiohara M, Kojima S, Takihara Y, Kobayashi M
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Journal Title
J Med Genet 45
Pages: 802-807
Peer Reviewed
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[Journal Article] Polycomb-group complex 1 acts as an E3 ubiquitin ligase for Geminin to sustain hematopoietic stem cell activity2008
Author(s)
Ohtsubo M, Yasunaga S, Ohno Y, Tsumura M, Okada S, Ishikawa N, Shirao K, Kikuchi A, Nishitani H, Kobayashi M, Takihara Y
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Journal Title
Proc Natl Acad Sci USA 105
Pages: 10396-10401
Peer Reviewed
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[Journal Article] Development of a new enzymatic diagnosis method for very-long-chain acyl-CoA dehydrogenase deficiency by detecting 2-hexadecenoyl-CoA production and its application in tandem mass spectrometry-based selective screening and newborn screening in Japan2008
Author(s)
Tajima G, Sakura N, Shirao K, Okada S, Tsumura M, Nishimura Y, Ono H, Hasegawa Y, Hata I, Naito E, Yamaguchi S, Shigematsu Y, Kobayashi M
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Journal Title
Pediatr Res 64
Pages: 667-672
Peer Reviewed
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