| Project/Area Number |
01570523
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| Research Category |
Grant-in-Aid for General Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | Nagoya University School of Medicine |
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Principal Investigator |
MATSUOKA Hiroshi Nagoya University Pediatrics, 医学部, 助手 (50157278)
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| Co-Investigator(Kenkyū-buntansha) |
山田 政功 名古屋大学, 医学部, 助手 (30200744)
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| Project Period (FY) |
1989 – 1990
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| Project Status |
Completed (Fiscal Year 1990)
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| Budget Amount *help |
¥2,100,000 (Direct Cost: ¥2,100,000)
Fiscal Year 1990: ¥1,100,000 (Direct Cost: ¥1,100,000)
Fiscal Year 1989: ¥1,000,000 (Direct Cost: ¥1,000,000)
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| Keywords | Primary immunodeficiency / Precursor B cell line / Abnormal DNA rearrangement / Recombinase / Severe combined immunodeficiency / X-Linked agammaglobulinemia / Common variable immunodeficiency / 伴性無ガンマグロブリン血症 / Common variable immunodeficiency / 重症複合型免疫不全症 / common variable immunodeficiency |
|---|
| Research Abstract |
1). EB virus transformed (precursor) B cell lines (139 cell lines) were established from 8 Severe Combined immunodeficiency (SCID) patients, one X-linked agammaglobulinemia (XLA) patient and 8 common variable immunodeficiency (CVID) patients.
2). Fourteen of 139 cell lines had precursor B cell phenotypes, shown by immunofluorescent technique.
3). The study of DNA rearrangements of Ig heavy chain gene loci in these B cell lines showed a defect of DJ-VDJ rearrangement in one XLA and one CVID patient, and showed a defect of DJ rearrangement in SCID patient.
4). RAG-1 mRNA was detected in SCID 86730 cell clone, which had DJ rearrangement defect suggesting of the abnormal recombinase system, by using RT-PCR method.
5). Recombinant vectors consisted of signal sequences, 12/23 spacer, Neo gene (inverted), HygroB gene and SRalpha promoter were constructed and used to measure recombinase activities of these B cell lines.
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