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Relationship between the expression of complement regulatory proteins and PIG-A gene abnormalities in CD34^+ cells of PNH.

Research Project

Project/Area Number 06671104
Research Category

Grant-in-Aid for General Scientific Research (C)

Allocation TypeSingle-year Grants
Research Field Hematology
Research InstitutionFukushima Medical College

Principal Investigator

SHICHISHIMA Tsutomu  Fukushima Medical College, Department of Medicine, Assistant Professor, 医学部, 講師 (10192105)

Co-Investigator(Kenkyū-buntansha) TERASAWA Takashi  Ohu University, Department of Dentistry, Associated Professor, 歯学部, 助教授 (40083422)
MARUYAMA Yukio  Fukushima Medical College, Department of Medicine, Professor, 医学部, 教授 (90004712)
Project Period (FY) 1994 – 1995
Project Status Completed (Fiscal Year 1995)
Budget Amount *help
¥1,800,000 (Direct Cost: ¥1,800,000)
Fiscal Year 1995: ¥1,400,000 (Direct Cost: ¥1,400,000)
Fiscal Year 1994: ¥400,000 (Direct Cost: ¥400,000)
KeywordsParoxysmal nocturnal hemoglobinuria (PNH) / Glycosylphosphatidylinositol (GPI)-anchored membrane proteins / CD55 / CD59 / Phosphatidylinositol glycan-class A (PIG-A) gene / CD34^+ cells / Flow cytometry / Phenotype / 発作性夜間血色素尿症(PNH) / PIG-A / 多核好中球 / Tリンパ球 / 顆粒球 / 補体防御膜蛋白
Research Abstract

We investigated the expression of glycosylphosphatidylinositol (GPI)-anchored membrane proteins (CD55 and CD59) by erythrocytes, platelets, granulocytes, erythroblasts, T and B lymphocytes, and CD34^+ cells from patients with paroxysmal nocturnal hemoglobinuria (PNH) and healthy volunteers using flow cytometric two-color analysis. In addition, we also studied the abnormality of phosphatidylinositol glycan-class A (PIG-A) gene derived from granulocytes, Tlymphocytes, and CD34^+ cells in the patients. The results are as follows :
1. We found that the expression of CD55 and CD59 by various cells as described above was discordant in PNH patients. In particular, we studied relationship between the phenotypes of circulating erythrocytes and cultured erythroblasts in the patients. Our results showed that the cultured erythroblasts in all the patients consisted of PNH I,II,and III erythroblasts or PNH II and III erythroblasts in spite of the final phenotype of mature erythrocytes. These finding … More s suggest that the final phenotype of erythrocytes in PNH is determined during maturation from erythroblasts to erythrocytes by the disappearance or persistence of PNH II erythroblasts (Shichishima et al., Blood, In press).
2. We investigated PIG-A gene abnormalities in peripheral blood granulocytes and T lymphocytes obtained from PNH patients by PCR and RT-PCR followed by nucleotide sequence analysis. Our present study focused on the relationship between the phenotypes of these cells detected by flow cytometric analysis and proportion of abnormal clone of the PIG-A gene. Our results suggest that PIG-A gene abnormalities can not prescribe the phenotypes of granulocytes from PNH patients but the volume of GPI-core, although a defect of PIG-A gene certainly underlies the occurence of PNH (Noji, Shichishima et al., Blood 86 : 132a, 1995).
3. One-color flow cytometric analysis showed that the CD34^+ cells derived from bone marrow blood from healthy volunteers consisted of a single population positive for CD59, while those from PNH patients had a continuous population from negative to positive for CD59. Moreover, we found that PIG-A gene abnormalities in bone marrow blood CD34^+ cells were same as those in peripheral blood granulocytes obtained from patients with PNH.We will have to evaluate carefully whether the CD34^+ cells could have the above two abnormalities of PIG-A gene in PNH. Less

Report

(3 results)
  • 1995 Annual Research Report   Final Research Report Summary
  • 1994 Annual Research Report
  • Research Products

    (15 results)

All Other

All Publications (15 results)

  • [Publications] 七島 勉: "発作性夜間血色素尿症(PNH)の病態" 血液・腫瘍科. 29. 441-454 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1995 Final Research Report Summary
  • [Publications] 野地秀義: "入院時Ham試験および砂糖水試験が陰性であった発作性夜間血色素尿症の1症例" 日本臨床血液学会誌. 35. 898-901 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1995 Final Research Report Summary
  • [Publications] Tsutomu Shichishima: "Glycosylphosphatidylinositol(GPI)-anchored membrane protein in clinical pathophysiology of paroxysmal nocturnal hemoglobinuria(PNH)" Fukushima Journal of Medical Science. 41. 1-13 (1995)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1995 Final Research Report Summary
  • [Publications] Tsutomu Shichishima: "In vivo effects of various therapies on complement-sensitive erythrocytes in paroxysmal nocturnal hemoglobinuria" International Journal of Hematology. 63. 291-302 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1995 Final Research Report Summary
  • [Publications] Tsutomu Shichishima: "Relationship between the phenotypes of circulating enythrocytes and cultured erythroblasts in paroxysmal nocturnal hemoglobinuria" Blood. (In press). (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1995 Final Research Report Summary
  • [Publications] Tsutomu Shichishima: "Pathophysiology of paroxysmal nocturnal hemoglobinuria (PNH)" Hemotology & Oncology. 29. 441-454 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1995 Final Research Report Summary
  • [Publications] Hideyoshi Noji, Tsutomu Shichishima et al.: "Negative Ham's test and sugar water test on admission in a PNH patient." Japanese Journal of Clinical Hematology. 35. 898-901 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1995 Final Research Report Summary
  • [Publications] Tsutomu Shichishima: "Glycosylphosphatidylinositol (GPI)-anchored membrane protein in clinical pathophysiology of paroxysmal nocturnal hemoglobinuria (PNH)" Fukushima Journal of Medical Scinence. 41. 1-13 (1995)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1995 Final Research Report Summary
  • [Publications] Tsutomu Shichishima et al.: "In vivo effects of various therapies on complement-sensitive erythrocytes in paroxysmal nocturnal hemoglobinuria." International Journal of Hematology. 63. 291-302 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1995 Final Research Report Summary
  • [Publications] Tsutomu Shichishima et al.: "Relationship between the phenotypes of circulating erythrocytes and cultured erythroblasts in paroxysmal nocturnal hemoglobinuria." Blood. (in press.). (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1995 Final Research Report Summary
  • [Publications] Tsutomu Shichishima: "In vivo effects of various therapies on complement-sensitive erythrocytes in paroxysmal nocturnal hemoglobinuria." International Journal of Hematology. (1996)

    • Related Report
      1995 Annual Research Report
  • [Publications] 七島 勉: "発作性夜間血色素尿症(PNH)の病態" 血液・腫瘍科. 29. 441-454 (1994)

    • Related Report
      1994 Annual Research Report
  • [Publications] 野地 秀義: "入院時Ham試験および砂糖水試験が陰性であった発作性夜間血色素尿症の1症例" 日本臨床血液学会雑誌. 35. 898-901 (1994)

    • Related Report
      1994 Annual Research Report
  • [Publications] 七島 勉: "特集・内科疾患と診断基準・病型分類・重症度/診断メモ PNH" 内科. 75. (1995)

    • Related Report
      1994 Annual Research Report
  • [Publications] Tsutomu Shichishima: "Glycosylphosphatidylinositol(GPI)-anchored membrane protein in clinical pathophysiology of paroxysmal nocturnal hemoglobinuria(PNH)" Fukushima Journal of Medical Science. 41. (1995)

    • Related Report
      1994 Annual Research Report

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Published: 1994-04-01   Modified: 2016-04-21  

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