Delaunay J
Laboratoire de Génétique Moléculaire Humaine (CNRS URA 1171), Institut Pasteur de Lyon, France.
FEBS Lett. 1995 Aug 1;369(1):34-7. doi: 10.1016/0014-5793(95)00460-q.
The red cell membrane is comprised of a lipid bilayer studded with transmembrane proteins, and laminated by a protein network, the membrane skeleton, at the surface of the inner monolayer. The erythrocyte owes its mechanical properties to the membrane skeleton. Hereditary spherocytosis, hereditary elliptocytosis or poikilocytosis, Southeast Asian ovalocytosis are hereditary hemolytic anemias, due to mutations in the genes encoding ankyrin, the anion exchanger, spectrin, protein 4.1 or protein 4.2, which are main proteins of the membrane. Recent advances in the field have led to fundamental questions.
红细胞膜由镶嵌有跨膜蛋白的脂质双层组成,并在内侧单层表面由蛋白质网络(膜骨架)分层。红细胞的机械特性归因于膜骨架。遗传性球形红细胞增多症、遗传性椭圆形红细胞增多症或异形红细胞症、东南亚椭圆形红细胞增多症是遗传性溶血性贫血,归因于编码锚蛋白、阴离子交换蛋白、血影蛋白、蛋白4.1或蛋白4.2的基因突变,这些是膜的主要蛋白质。该领域的最新进展引发了一些基本问题。