Document Detail

Clinical severity of β-thalassaemia/Hb E disease is associated with differential activities of the calpain-calpastatin proteolytic system.
MedLine Citation:
PMID:  22615919     Owner:  NLM     Status:  MEDLINE    
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.
Suriyan Sukati; Saovaros Svasti; Roberto Stifanese; Monica Averna; Nantika Panutdaporn; Tipparat Penglong; Edon Melloni; Suthat Fucharoen; Gerd Katzenmeier
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2012-05-16
Journal Detail:
Title:  PloS one     Volume:  7     ISSN:  1932-6203     ISO Abbreviation:  PLoS ONE     Publication Date:  2012  
Date Detail:
Created Date:  2012-05-22     Completed Date:  2013-01-08     Revised Date:  2013-06-24    
Medline Journal Info:
Nlm Unique ID:  101285081     Medline TA:  PLoS One     Country:  United States    
Other Details:
Languages:  eng     Pagination:  e37133     Citation Subset:  IM    
Institute of Molecular Biosciences, Mahidol University, Salaya Campus, Nakorn Pathom, Thailand.
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MeSH Terms
Antigens, CD34 / metabolism
Calcium-Binding Proteins / metabolism*,  pharmacology
Calpain / antagonists & inhibitors*,  metabolism*
Case-Control Studies
Cell Differentiation / drug effects
Cells, Cultured
Erythroid Precursor Cells / drug effects,  metabolism
Proteolysis / drug effects
Recombinant Proteins / pharmacology
Severity of Illness Index
alpha-Globins / metabolism
beta-Thalassemia / enzymology*,  pathology*
Reg. No./Substance:
0/Antigens, CD34; 0/Calcium-Binding Proteins; 0/Recombinant Proteins; 0/alpha-Globins; 79079-11-1/calpastatin; EC 3.4.22.-/Calpain; EC 3.4.22.-/mu-calpain

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