Harlow R W, Lowenthal R M
Br J Haematol. 1982 Jan;50(1):35-41. doi: 10.1111/j.1365-2141.1982.tb01888.x.
Analysis of the erythrocyte membrane protein of an atypical case of congenital dyserythropoietic anaemia type II (CDA II) by electrophoresis on polyacrylamide gels revealed marked abnormalities. One-dimensional analysis showed a pronounced decrease in levels of B1.1 components, an increase in the level of B1.4 and the appearance of new components in the A region as well as in the C region of the gel. There were no artefacts due either to the presence of early red cells or to abnormally high levels of proteolytic enzyme activity in the CDA II preparations. Two-dimensional analysis confirmed the alterations in membrane components showing two novel A region species not reported in other studies of this disease. Abnormalities in components of such large molecular size may explain the greater degree of membrane perturbation seen in the present case and support the idea that CDA II may embrace more than one disease entity.
通过聚丙烯酰胺凝胶电泳对一例非典型II型先天性红细胞生成异常性贫血（CDA II）的红细胞膜蛋白进行分析，结果显示存在明显异常。一维分析表明，B1.1成分水平显著降低，B1.4水平升高，并且在凝胶的A区和C区出现了新的成分。CDA II制剂中不存在因早期红细胞的存在或蛋白水解酶活性异常高而导致的假象。二维分析证实了膜成分的改变，显示出在该疾病的其他研究中未报道的两种新的A区成分。如此大分子量成分的异常可能解释了本病例中观察到的更大程度的膜扰动，并支持CDA II可能包含不止一种疾病实体的观点。
