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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
Roberto Stifanese
Monica Averna
Nantika Panutdaporn
Tipparat Penglong
Edon Melloni
Suthat Fucharoen
Gerd Katzenmeier
Mahidol University
Universita degli Studi di Genova
Keywords: Agricultural and Biological Sciences;Biochemistry, Genetics and Molecular Biology;Medicine
Issue Date: 16-May-2012
Citation: PLoS ONE. Vol.7, No.5 (2012)
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 β 0 -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. © 2012 Sukati et al.
ISSN: 19326203
Appears in Collections:Scopus 2011-2015

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