Publication: Globin chain turnover in reticulocytes from patients with β°‐thalassaemia/Hb E disease
Issued Date
1995-01-01
Resource Type
ISSN
16000609
09024441
09024441
Other identifier(s)
2-s2.0-0028886676
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Mahidol University
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SCOPUS
Bibliographic Citation
European Journal of Haematology. Vol.55, No.5 (1995), 322-326
Suggested Citation
R. W. Kalpravidh, S. Komolvanich, P. Wilairat, S. Fucharoen Globin chain turnover in reticulocytes from patients with β°‐thalassaemia/Hb E disease. European Journal of Haematology. Vol.55, No.5 (1995), 322-326. doi:10.1111/j.1600-0609.1995.tb00704.x Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/17476
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Title
Globin chain turnover in reticulocytes from patients with β°‐thalassaemia/Hb E disease
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Abstract
β°‐Thalassaemia/Hb E disease is the most frequent β‐thalassaemia in Thailand. However, patients have a varying degree of anaemia. The difference in severity is attributed to a differential accumulation of unpaired α‐globin chains, which is the net result of biosynthesis and catabolism. Turnover of newly synthesized globin chains in reticulocytes from β°‐thalassaemia/Hb E patients was determined. Proteolysis was ATP‐independent and degraded only 10–15% of the radiolabelled globin during a 4‐h incubation period at 37 °C and there was no difference in globin turnover in reticulocytes from subjects with mild and severe forms of β°‐thalassaemia/Hb E. These results indicate that excess α‐globin chains do not act as substrates for the reticulocyte proteolytic system and that the difference in severity of anaemia in β°‐thalassaemia/Hb E disease cannot be explained by a difference in selective post‐translational catabolism. © Munksgaard 1995