Etiology of congenital agammaglobulinemia: Growth defect of precursor B lymphoblastoid cell lines and immunoglobulin gene rearrangements.
| Project/Area Number |
62480224
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| Research Category |
Grant-in-Aid for General Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | TOHOKU UNIVERSITY |
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Principal Investigator |
TSUCHIYA Shigeru Research Institute for Tuberculosis and Cancer, Tohoku University, 抗酸菌病研究所, 助教授 (30124605)
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| Co-Investigator(Kenkyū-buntansha) |
寺沢 政彦 東北大学, 抗酸菌病研究所, 助手 (80192201)
SATO Tetsuo Research Institute for Tuberculosis and Cancer, Tohoku University, 抗酸菌病研究所, 助手 (90170761)
KONNO Tasuke Research Institute for Tuberculosis and Cancer, Tohoku University, 抗酸菌病研究所, 教授 (00004846)
TERASAWA Masahiko Research Institute for Tuberculosis and Cancer, Tohoku University
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| Project Period (FY) |
1987 – 1988
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| Project Status |
Completed (Fiscal Year 1988)
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| Budget Amount *help |
¥6,200,000 (Direct Cost: ¥6,200,000)
Fiscal Year 1988: ¥2,000,000 (Direct Cost: ¥2,000,000)
Fiscal Year 1987: ¥4,200,000 (Direct Cost: ¥4,200,000)
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| Keywords | congenital agammaglobulinemia / Precursor B cell line / IgD / モノクローナル抗体 / IgD / 細胞クローニング / K+λ産生細胞クローン / K+入鎖産生細胞クローン / EBウイルス / プレBリンパ芽球様細胞株 / 表面マーカー |
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| Research Abstract |
Epstein-Barr virus (EBV) induced B lymphoblastoid cell lines (B-LCL) were established from bone marrow cells of the patients with congenital agammaglobulinemia (CAG).We found several immature B-LCL, some of which possess no cell-surface and cytoplastic immunoglobulins, or possess only cytoplasmic heavy chains. We examined the mode of expression of B-cell associated antigens on EBV-induced B-LCL and failed to detect any CAG specific and developmental stage-specific expression of known B-cell related antigens on those B-LCL. These facts indicate that expression of B cell associated antigens on EBV-induced B-LCL does not depend on the developmental stage of B cells, rather depend on the state of EBV infections.
If we can detect LCL which express immunoglobulins with very unusual manner, those LCL will probably give us oppotunities to elucidate the etiology of CAG. Fortunately we found 2 such cell lines, K5 and K4, from the same CAG patient. K5 was very unique because they expressed only IgD (delea,lambda) on the surface and produced IgD. K4 was also very unique because they produced lambda heavy chain, and both kappa and lambda light chains. Cell cloning experiments repeated 3 times definitely confirmed that K4 cells produced both kappa and lambda light chains with single mu chains. The pattern of immunoglobolin production seen on K4 and K5 was probably the first ones in the literature. We are going to clone immunoglobuline genes of K4 and K5, and to elucidate the genetic mechanism of the expression of unusual immunoglobulins.
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Report
(3 results)
Research Products
(16 results)
