1996 Fiscal Year Final Research Report Summary
Mutation of PIG-A gene during differentiation from hematopoietic progenitors in paroxysmal nocturnal hemoglobinuria
| Project/Area Number |
07671226
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Hematology
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| Research Institution | Hyogo College of Medicine |
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Principal Investigator |
KANAMARU Akihisa Hyogo College of Medicine, Department of Medicine Associate Professor, 医学部, 助教授 (70068534)
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| Project Period (FY) |
1995 – 1996
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| Keywords | PNH / PIG-A gene / hematopoietic progenitors / GPI-anchor / CD59 |
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| Research Abstract |
Recent studies indicate that the GPI-anchor biosynthesis is associated with PIG-A gene. A variety of mutation (s) of this gene has been found in every patients with paroxysmal nocturnal hemoglobinuria (PNH). We studied on the mutation of PIG-A gene during the differentiation from hematopoietic progen itors in PNH.Non-phagocytic mononuclear cells were prepared from the patients with PNH after obtaining the informed consent, and cultured in methylcellulose for 10-14 days. Individual colonies/bursts were picked up and divided into two fractions for the analyzes of CD59 expression and PIG-A gene status. We have obtained 4 groups of colonies/bursts ; each group of CD59+with normal PIG-A,CD59+with mutated PIG-A,CD59- with normal PIG-A,and CD59- with mutated PIG-A.A group of CD59+ with normal PIG-A and that of CD59- with mutated PIG-A might be normal and the PNH clone, respectivelly. Mutation (s) may be found in other region of the gene for the colonies/bursts of CD59- with normal PIG-A group. However, there is a discordance between the expression of GPI-anchored proteins and PIG-A mutation in the group of CD59+ with mutated PIG-A.It is speculated that another pathway bypassing PIG-A gene is in operation for the GPI-anchor biosynthesis.
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