1993 Fiscal Year Final Research Report Summary
Study of bone maroow transplantation for lysosomal storage diseases.
| Project/Area Number |
04670625
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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 | Nihon University |
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Principal Investigator |
SAKIYAMA Takeshi Nihon University School of Medicine, Dep, of Pediatrics, Assistant Professor, 医学部, 講師 (20130510)
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| Co-Investigator(Kenkyū-buntansha) |
YOSHIDA Yasuyoshi Nihon University School of Medicine, Dep of Pediatrics, Instructor., 医学部, 助手 (60240321)
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| Project Period (FY) |
1992 – 1993
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| Keywords | Niemann-Pick / BMT / Sry / 動物モデル / Sry |
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| Research Abstract |
Bone marrow transplantation is the most promising therapy fo lysosomal storage disease, although variable CNS outcomes have been reported. The colonization and proliferation along the recipient vessels were demonstrated in our prev-ious study using Niemann-Pick mice bone marrow, cells in normal recepient mice (Sakiyama et al 1986). A similar phenomenon is postulated in CNS, even though there is the presence of the blood-brain barrier, which may or may not be an impediment to monocyte diapedesis.
In the present study, detection of Y-specific target sequence in the bone-marrow-transplanted tissues, especially in the brain, is accomplished by the PCR method. We have proved by PCR methods using Sry and Zfy genes the colonization and proliferation of a male mononuclear phagocyte system (microglia) both in cerebrum and cerebellum even after 5 weeks transplantation. Also, donor cell colonization and proliferation in the recepient's CNS, following bone marrow transplantation (BMT), have been demonstrated by in-situ hybrization using a synthesized Y chromosome specific repeated sequence.
These results show that circulating macrophages derived from donor bone marrow cells settle in and colonize the brain parenchyma as well as visceral organs.
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