Identification and functional analysis of the genes critical for B cell terminal differentiation in ICF syndrome.
Project/Area Number |
25670484
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Research Category |
Grant-in-Aid for Challenging Exploratory Research
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Allocation Type | Multi-year Fund |
Research Field |
Pediatrics
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Research Institution | National Defense Medical College |
Principal Investigator |
NONOYAMA Shigeaki 防衛医科大学校(医学教育部医学科進学課程及び専門課程、動物実験施設、共同利用研究, 医学教育部医学科専門課程, 教授 (40280961)
|
Project Period (FY) |
2013-04-01 – 2015-03-31
|
Project Status |
Completed (Fiscal Year 2014)
|
Budget Amount *help |
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2014: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2013: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
|
Keywords | 小児免疫 / アレルギー / 膠原病学 |
Outline of Final Research Achievements |
ICF syndrome is characterized by immunodeficiency and a genetic disorder caused by aberrant DNA methylation. Its immunodeficiency is known to be derived from disability of B cells maturation. So, we have tried to compare the methylation status of B cells of ICF syndrome patients and healthy controls. As a result, it was possible to clarify the group of genes dramatically shows the difference. Also, we have tried to compare the methylation status of immature B cells and mature B cells in healthy controls. As a result the difference has become to be also clear. We have also tried to do RNA analysis. As a result the extent of gene expression has become also clear. By combining these results of three analyses, we succeeded to narrow down genes that are closely responsible for the maturation of B cells.
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Report
(3 results)
Research Products
(18 results)
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[Journal Article] The soluble ST2 and NT-proBNP combination, a useful biomarker for predicting prognosis of childhood pulmonary arterial hypertension.2014
Author(s)
Chida A, Sato H, Shintani M, Nakayama T, Kawamura Y, Furutani Y, Inai K, Saji T, Matsuoka R, Nonoyama S, Nakanishi T.
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Journal Title
Circ J.
Volume: 78
Pages: 436-442
Related Report
Peer Reviewed
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[Journal Article] Galectin-9 enhances cytokine secretion, but suppresses survival and degranulation, in human mast cell line2014
Author(s)
Kojima R, Ohno T, Iikura M, Niki T, Hirashima M, Iwaya K, Tsuda H, Nonoyama S, Matsuda A, Saito H, Matsumoto K, Nakae S
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Journal Title
PLoS ONE
Volume: 9
Issue: 1
Pages: e86106-e86106
DOI
Related Report
Peer Reviewed
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[Journal Article] Mutations of NOTCH3 in childhood pulmonary arterial hypertension.2014
Author(s)
Chida A, Shintani M, Matsushita Y, Sato H, Eitoku T, Nakayama T, Furutani Y, Hayama E, Kawamura Y, Inai K, Ohtsuki S, Saji T, Nonoyama S, Nakanishi T.
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Journal Title
Mol Genet Med.
Volume: 印刷中
Related Report
Peer Reviewed
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[Journal Article] Efficacy and Safety of IgPro20, a Subcutaneous Immunoglobulin, in Japanese Patients with Primary Immunodeficiency Diseases.2014
Author(s)
Kanegane H, Imai K, Yamada M, Takada H, Ariga T, Bexon M, Rojavin M, Hu W, Kobayashi M, Lawo JP, Nonoyama S, Hara T, Miyawaki T.
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Journal Title
J Clin Immunol.
Volume: 34
Pages: 204-211
Related Report
Peer Reviewed
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[Journal Article] Primary immunodeficiency diseases: an update on the classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency.2014
Author(s)
Al-Herz W, Bousfiha A, Casanova JL, Chatila T, Conley ME, Cunningham-Rundles C, Etzioni A, Franco JL, Boby Gaspar H, Holland SM, Klein C, Nonoyama S, Ochs HD,
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Journal Title
Front Immunol.
Volume: 162
Pages: 1-33
Related Report
Peer Reviewed
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[Journal Article] Common variable immunodeficiency classification by quantifying T-cell receptor and immunoglobulin κ-deleting recombination excision circles.2013
Author(s)
Kamae C, Nakagawa N, Sato H, Honma K, Mitsuiki N, Ohara O, Kanegane H, Pasic S, Pan-Hammarstrom Q, van Zelm MC, Morio T, Imai K, Nonoyama S.
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Journal Title
J Allergy Clin Immunol.
Volume: 131
Pages: 1437-1440
Related Report
Peer Reviewed
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[Journal Article] Neonatal herpes encephalitis caused by a virologically confirmed acyclovir resistant herpes simplex virus type 1.2013
Author(s)
Kakiuchi S, Nonoyama S, Wakamatsu H, Kogawa K, Wang L, Kinoshita-Yamaguchi H, Takayama-Ito M, Lim CK, Inoue N, Mizuguchi M, Igarashi T, Saijo M.
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Journal Title
J. Clin. Microbiol.
Volume: 51
Pages: 356-359
Related Report
Peer Reviewed
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[Journal Article] Impaired KLHL3-Mediated Ubiquitination of WNK4 Causes Human Hypertension.2013
Author(s)
Wakabayashi M, Mori T, Isobe K. Sohara E, Susa K, Araki Y, Chiga M, Kikuchi E, Nomura N, Mori Y, Matsuo H, Murata T, Nomura S, Asano T, Kawaguchi H, Nonoyama S, Rai T, Sasaki S, Uchida S.
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Journal Title
Cell Reports.
Volume: 3
Pages: 858-868
Related Report
Peer Reviewed
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[Journal Article] A phenotypic approach for IUIS PID classification and diagnosis: guidelines for clinicians at the bedside.2013
Author(s)
Bousfiha AA, Jeddane L, Ailal F, Al Herz W, Conley ME, Cunningham-Rundles C, Etzioni A, Fischer A, Franco JL, Geha RS, Hammarström L, Nonoyama S, Ochs HD, Roifman CM, Seger R, Tang ML, Puck JM, Chapel H, Notarangelo LD, Casanova JL.
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Journal Title
J Clin Immunol.
Volume: 33
Pages: 1078-1087
Related Report
Peer Reviewed
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[Journal Article] Development of enzyme-linked immunosorbent assays for urinary thiazide-sensitive Na-Cl cotransporter (NCC) measurement.2013
Author(s)
Isobe K, Mori T, Asano T, Kawaguchi H, Nonoyama S, Kumagai N, Kamada F, Morimoto T, Hayashi M, Sohara E, Rai T, Sasaki S, Uchida S.
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Journal Title
Am J Physiology Renal Physiology.
Volume: 305
Pages: 1374-1381
Related Report
Peer Reviewed
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[Journal Article] Chronic Granulomatous Disease: Two Decades of Experience From a Tertiary Care Centre in North West India.2013
Author(s)
Rawat A, Singh S, Suri D, Gupta A, Saikia B, Minz RW, Sehgal S, Vaiphei K, Kamae C, Honma K, Nakagawa N, Imai K, Nonoyama S, Oshima K, Mitsuiki N, Ohara O,
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Journal Title
J Clin Immunol.
Volume: 33
Pages: 857-864
Related Report
Peer Reviewed
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