You are currently on the new version of our website. Access the old version .
Thalassemia ReportsThalassemia Reports
  • Thalassemia Reports is published by MDPI from Volume 12 Issue 1 (2022). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.
  • Article
  • Open Access

29 September 2014

Differential Regulation of Plasma Proteins between Members of a Family with Homozygous HbE and HbEβ-thalassemia

,
,
,
and
1
Biophysics & Structural Genomics Division, Saha Institute of Nuclear Physics, Kolkata 700064, India
2
Institute of Genetic Medicine and Genomic Science, Kolkata 700064, India
3
Crstallography and Molecular Biology Division, Saha Institute of Nuclear Physics, Kolkata 700064, India
*
Author to whom correspondence should be addressed.

Abstract

In this report we’ve compared the plasma protein profiles of 4 individuals in a family. Father and the younger son both are hemoglobin (Hb) Eβ-thalassemic {Cod 26 (G-A)/IVS 1- 5 (G-C)}, but the father never requires transfusion, whereas the younger son requires monthly blood transfusion. Mother and the elder son are HbEE {Cod 26 (G-A)/Cod 26 (GA)} without any history of transfusion. Proteomic study was done on the plasma fraction of the blood following ammonium sulphate precipitation. Proteins were separated by 2D-gel electrophoresis, expression of proteins compared by densitometry and proteins identified by tandem MALDI mass spectrometry. Proteins responsible in hemolysis, hypercoagulation and hemoglobin scavenging have shown differential regulation, establishing the relation between the differences in the levels of plasma proteins with the progression of the disease phenotype, manifested in the extent of transfusion dependence of the patient.

Article Metrics

Citations

Article Access Statistics

Multiple requests from the same IP address are counted as one view.