The genetic defect of PNH
نویسندگان
چکیده
منابع مشابه
The PIG-anchoring defect in NK lymphocytes of PNH patients.
Paroxysmal nocturnal hemoglobinuria (PNH) is clinically characterized by intravascular hemolysis, hemoglobinuria, iron deficiency anemia, and venous thrombosis. Pathophysiologically the disease has now been generally accepted as an acquired defect of phosphatidylinositol glycan (PIG)-anchored molecules on the cell surface of bone marrow-derived cells. This defect is functionally characterized b...
متن کاملCorrection of the PNH defect by GPI-anchored protein transfer.
Hemolytic anemia is a major feature of paroxysmal nocturnal hemoglobinuria (PNH). Intravascular red blood cell (RBC) destruction is caused by increased sensitivity of the abnormal erythrocyte to complement-mediated lysis, due to the GPI absence of a membrane-bound glycosylphosphatidylinositol (GPI)-linked protein, which functions as an inhibitor of reactive lysis (CD59). Both in vivo and in vit...
متن کاملphylogeography and genetic diversity of the lesser mouse- eared bat (myotis blythii) in iran
in current study, 63 samples of bat populations collected from differ regions were used for evaluating the geographic variations. twenty cranial and dental characters for traditional morphometric and landmarks method on the ventral, dorsal skull and mandible for geometry morphometric studies were used. statistical analyses of traditional morphometric and geometry morphometric data indicated low...
Correction of the PNH defect by GPI protein transfer: still an open question.
Sloand et al1 report that in human red blood cells (RBCs) the paroxysmal nocturnal hemoglobinuria (PNH) defect can be corrected by transfer of glycosylphosphatidylinositol (GPI)-anchored proteins to GPI-deficient cells. It has been demonstrated before by a number of authors that cell-to-cell transfer of GPI-anchored proteins can occur, and I have no doubt that a similar event also takes place b...
متن کاملCharacteristics of Taiwanese patients of PNH in the international PNH registry
BACKGROUND Paroxysmal nocturnal hemoglobinuria (PNH) is a rare and acquired hematopoietic stem cell disease, with florid clinical presentations. Although this disease has been characterized in the western countries, its clinical and laboratory features in Taiwan have not yet been reported. RESULTS As a part of an international prospective, non-interventional, observational registration trial ...
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ژورنال
عنوان ژورنال: Clinical & Experimental Immunology
سال: 2008
ISSN: 0009-9104,1365-2249
DOI: 10.1111/j.1365-2249.1994.tb06253.x