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Please use this identifier to cite or link to this item: http://repository.li.mahidol.ac.th/dspace/handle/123456789/1805
Title: Clinical severity of β-thalassaemia/Hb E disease is associated with differential activities of the calpain-calpastatin proteolytic system
Authors: Suriyan Sukati
Saovaros Svasti
Stifanese, Roberto
Averna, Monica
Nantika Panutdaporn
Tipparat Penglong
Melloni, Edon
Suthat Fucharoen
Katzenmeier, Gerd
Mahidol University. Institute of Molecular Biosciences
Mahidol University. Faculty of Science. Department of Biochemistry
Keywords: Clinical Severity;b-thalassaemia/Hb E;Disease;Associated;Differential;Activities;Calpain-Calpastatin;Proteolytic;Open Access article
Issue Date: May-2012
Citation: Plos One. Vol.7, No.5 (2012), 1-8
Abstract: Earlier observations in the literature suggest that proteolytic degradation of excess unmatched α-globin chains reduces their accumulation and precipitation in β-thalassaemia erythroid precursor cells and have linked this proteolytic degradation to the activity of calpain protease. The aim of this study was to correlate the activity of calpain and its inhibitor, calpastatin, with different degrees of disease severity in β-thalassaemia. CD34+ cells were enriched from peripheral blood of healthy individuals (control group) and patients with mild and severe clinical presentations of β0-thalassaemia/Hb E disease. By ex vivo cultivation promoting erythroid cell differentiation for 7 days, proerythroblasts, were employed for the functional characterization of the calpain-calpastatin proteolytic system. In comparison to the control group, enzymatic activity and protein amounts of μ-calpain were found to be more than 3-fold increased in proerythroblasts from patients with mild clinical symptoms, whereas no significant difference was observed in patients with severe clinical symptoms. Furthermore, a 1.6-fold decrease of calpastatin activity and 3.2-fold accumulation of a 34 kDa calpain-mediated degradation product of calpastatin were observed in patients with mild clinical symptoms. The increased activity of calpain may be involved in the removal of excess α-globin chains contributing to a lower degree of disease severity in patients with mild clinical symptoms.
URI: http://repository.li.mahidol.ac.th/dspace/handle/123456789/1805
metadata.dc.identifier.url: http://www.plosone.org/article/fetchObject.action?uri=info:doi/10.1371/journal.pone.0037133&representation=PDF
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