التفاصيل البيبلوغرافية
| العنوان: |
The Role of T Lymphocytes in the Pathogenesis of Paroxysmal Nocturnal Hemoglobinuria |
| المؤلفون: |
Chenyuan Li, Xifeng Dong, Huaquan Wang, Zonghong Shao |
| المصدر: |
Frontiers in Immunology, Vol 12 (2021) |
| بيانات النشر: |
Frontiers Media S.A., 2021. |
| سنة النشر: |
2021 |
| المجموعة: |
LCC:Immunologic diseases. Allergy |
| مصطلحات موضوعية: |
paroxysmal nocturnal hemoglobinuria, aplastic anemia, T lymphocytes, pathogenesis, immune escape, Immunologic diseases. Allergy, RC581-607 |
| الوصف: |
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematopoietic stem cell genetic mutation disease that causes defective erythrocyte membrane hemolysis. Its pathologic basis is the mutation of the PIG-A gene, whose product is necessary for the synthesis of glycosylphosphatidylinositol (GPI) anchors; the mutation of PIG-A gene results in the reduction or deletion of the GPI anchor, which leads to the deficiency of GPI-anchored proteins (GPI-APs), such as CD55 and CD59, which are complement inhibitors. The deficiency of complement inhibitors causes chronic complement-mediated intravascular hemolysis of GPI-anchor-deficient erythrocyte. PIG-A gene mutation could also be found in bone marrow hematopoietic stem cells (HSCs) of healthy people, but they have no growth advantage; only the HSCs with PIG-A gene mutation in PNH patients have this advantage and expand. Besides, HSCs from PIG-A-knockout mice do not show clonal expansion in bone marrow, so PIG-A mutation cannot explain the clonal advantage of the PNH clone and some additional factors are needed; thus, in recent years, many scholars have put forward the theories of the second hit, and immune escape theory is one of them. In this paper, we focus on how T lymphocytes are involved in immune escape hypothesis in the pathogenesis of PNH. |
| نوع الوثيقة: |
article |
| وصف الملف: |
electronic resource |
| اللغة: |
English |
| تدمد: |
1664-3224 |
| Relation: |
https://www.frontiersin.org/articles/10.3389/fimmu.2021.777649/full; https://doaj.org/toc/1664-3224 |
| DOI: |
10.3389/fimmu.2021.777649 |
| URL الوصول: |
https://doaj.org/article/c66b4bbd71e0429ebd916bb6101f177e |
| رقم الانضمام: |
edsdoj.66b4bbd71e0429ebd916bb6101f177e |
| قاعدة البيانات: |
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