[Analysis of erythrocytic spectrin in thalassemia and in Lepore hemoglobinosis]. / Analisi della spettrina eritrocitaria nella talassemia e nell'emoglobinosi lepore.

Total proteins and red cell membrane spectrin were determined in normal subjects, patients with hyperhaemolytic and anhaemolytic thalassaemia, and Hb Lepore from a single family. Electrophoresis on acrylamide gels was performed after solubilisation of the material in SDS using the whole membrane and spectrin. Amino acid composition was also determined after hot acid hydrolysis. Resin chromatography was employed to recognise acid, neutral and basic aminoo acids, glucosamine, and galactosamine. It was found that the significant changes in spectrin amino acid composition observed in thalassaemic subjects with peripheral hyperhaemolysis were not apparent in anhaemolytic patients, nor in the heterozygote, clinically asymptomatic carriers of Hb Lepore. These changes are certainly of importance on account of the structural alterations noted in the spectrin of the subjects concerned.

[Erythrocyte changes of membrane proteins in some hemoglobinopathies]. / Alterazioni eritrocitarie delle proteine di membrana in alcune emoglobinopatie

The electrophoretic behaviour of red cell membrane proteins was studied in thalassaemia major and minor and in sickle-cell anaemia. Protein amino acid composition was also determined following hot hydrolysis of the stromas. Experiments with various solubilisation methods, electrophoresis supports and buffers showed certain changes in these three diseases, accompanied by alterations of the percent ratio of some amino acids.