Differential regulation of plasma proteins between members of a family with homozygous HbE and HbEβ-thalassemia
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...
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| Format: | Article |
| Language: | English |
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MDPI AG
2014-09-01
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| Series: | Thalassemia Reports |
| Subjects: | |
| Online Access: | http://www.pagepressjournals.org/index.php/thal/article/view/1837 |
| _version_ | 1797964252092301312 |
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| author | Suchismita Halder Tridip Chatterjee Amit Chakravarty Sudipa Chakravarty Abhijit Chakrabarti |
| author_facet | Suchismita Halder Tridip Chatterjee Amit Chakravarty Sudipa Chakravarty Abhijit Chakrabarti |
| author_sort | Suchismita Halder |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-11T01:41:08Z |
| format | Article |
| id | doaj.art-4cdc6d67425d411f8753c4a9496b6cca |
| institution | Directory Open Access Journal |
| issn | 2039-4357 2039-4365 |
| language | English |
| last_indexed | 2024-04-11T01:41:08Z |
| publishDate | 2014-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Thalassemia Reports |
| spelling | doaj.art-4cdc6d67425d411f8753c4a9496b6cca2023-01-03T08:20:42ZengMDPI AGThalassemia Reports2039-43572039-43652014-09-014210.4081/thal.2014.18373876Differential regulation of plasma proteins between members of a family with homozygous HbE and HbEβ-thalassemiaSuchismita Halder0Tridip Chatterjee1Amit Chakravarty2Sudipa Chakravarty3Abhijit Chakrabarti4Biophysics & Structural Genomics Division, Saha Institute of Nuclear Physics, KolkataInstitute of Genetic Medicine and Genomic Science, KolkataInstitute of Genetic Medicine and Genomic Science, KolkataInstitute of Genetic Medicine and Genomic Science, KolkataCrstallography and Molecular Biology Division, Saha Institute of Nuclear Physics, KolkataIn 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.http://www.pagepressjournals.org/index.php/thal/article/view/1837HbE-β thalassemia, blood transfusion, differential proteomics. |
| spellingShingle | Suchismita Halder Tridip Chatterjee Amit Chakravarty Sudipa Chakravarty Abhijit Chakrabarti Differential regulation of plasma proteins between members of a family with homozygous HbE and HbEβ-thalassemia Thalassemia Reports HbE-β thalassemia, blood transfusion, differential proteomics. |
| title | Differential regulation of plasma proteins between members of a family with homozygous HbE and HbEβ-thalassemia |
| title_full | Differential regulation of plasma proteins between members of a family with homozygous HbE and HbEβ-thalassemia |
| title_fullStr | Differential regulation of plasma proteins between members of a family with homozygous HbE and HbEβ-thalassemia |
| title_full_unstemmed | Differential regulation of plasma proteins between members of a family with homozygous HbE and HbEβ-thalassemia |
| title_short | Differential regulation of plasma proteins between members of a family with homozygous HbE and HbEβ-thalassemia |
| title_sort | differential regulation of plasma proteins between members of a family with homozygous hbe and hbeβ thalassemia |
| topic | HbE-β thalassemia, blood transfusion, differential proteomics. |
| url | http://www.pagepressjournals.org/index.php/thal/article/view/1837 |
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