赤血球膜蛋白異常の検出法としてのフローサイトメトリー： 発作性夜間ヘモグロビン尿症（PNH）の病態解析の基礎と応用

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  • Flow Cytometry as a Tool to Detect Defects in Red Blood Cell Membrane Proteins–Basis and Application for Analysis of Pathophysiology of Paroxysmal Nocturnal Hemoglobinuria
  • セッケッキュウマク タンパク イジョウ ノ ケンシュツホウ ト シテノ フロー サイトメトリー ホッサセイ ヤカン ヘモグロビン ニョウショウ PNH ノ ビョウタイ カイセキ ノ キソ ト オウヨウ

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Flow cytometry (FCM) is a useful tool to detect phenotypes of blood cells. Paroxysmal nocturnal hemoglobinuria　(PNH), an acquired hematopoietic stem cell disorder, is characterized by its affected blood cells defective in the　expression of glycosyl phosphatidylinositol (GPI)-anchored proteins. GPI-anchored membrane proteins on the red　blood cells (RBC) include two major complement-regulatory membrane proteins, CD55 and CD59. The most characteristic　clinical feature of PNH, intravascular hemolysis of PNH-affected RBC, is induced by the complement-mediated　lysis of CD55- and CD59-negative PNH-RBC. In addition to the RBC membrane defects, leukocytes and platelets　are also defective in the expression of GPI-anchored proteins, because of the PNH mutations occurred in the pluripotential　hematopoietic stem cells. The GPI-anchored proteins with cell-lineage specificity, CD16b in neutrophils and　CD14 in monocytes, are also shown to be deficient in PNH by FCM. We recently applied FCM to the demonstration　of CD59-deficient populations in reticulocytes. FCM is also useful to demonstrate RBC membrane alteration in the　aspects of lipids associated with the procoagulant pathophysiology in PNH.

収録刊行物

  • 膜

    膜 32 (3), 147-154, 2007

    日本膜学会

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