作者
Maria Alejandra Lizarralde Iragorri, Sara El Hoss, Valentine Brousse, Sophie D Lefevre, Michael Dussiot, Tieying Xu, Alexander Rodrigo Ferreira, Yann Lamarre, Ana Cristina Silva Pinto, Simone Kashima, Claudine Lapouméroulie, Dimas Tadeu Covas, Caroline Le Van Kim, Yves Colin, Jacques Elion, Olivier Français, Bruno Le Pioufle, Wassim El Nemer
发表日期
2018
期刊
Lab on a Chip
卷号
18
期号
19
页码范围
2975-2984
出版商
Royal Society of Chemistry
简介
The human red blood cell is a biconcave disc of 6–8 × 2 μm that is highly elastic. This capacity to deform enables it to stretch while circulating through narrow capillaries to ensure its main function of gas exchange. Red cell shape and deformability are altered in membrane disorders because of defects in skeletal or membrane proteins affecting protein–protein interactions. Red cell properties are also altered in other pathologies such as sickle cell disease. Sickle cell disease is a genetic hereditary disorder caused by a single point mutation in the β-globin gene generating sickle haemoglobin (HbS). Hypoxia drives HbS polymerisation that is responsible for red cell sickling and reduced deformability. The main clinical features of sickle cell disease are vaso-occlusive crises and haemolytic anaemia. Foetal haemoglobin (HbF) inhibits HbS polymerisation and positively impacts red cell survival in the circulation but the …
引用总数
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