A surprisingly high percentage of clinical complications affecting beta-thalassemia (beta-thal) patients is due to ectopic calcification. In these patients, elastic fiber calcification is associated with accumulation of anion superoxide and to increased levels of oxidized proteins and lipids. As a consequence, carboxylation of the calcification inhibitor Matrix Gla Protein (MGP) might be impaired. Independently from the gene defect, common pathogenic pathways are associated with ectopic calcification in PXE and in a number of beta-thal patients.
Oxidative stress is a key regulator of ectopic calcifications in beta-Thalassemia patients / Quaglino, Daniela; Boraldi, Federica; Annovi, Giulia; Cianciulli, Paolo. - In: THE FASEB JOURNAL. - ISSN 0892-6638. - ELETTRONICO. - 27:(2013), pp. 476-476. (Intervento presentato al convegno Experimental Biology 2013 tenutosi a Boston nel 20-24 Aprile 2013).
Oxidative stress is a key regulator of ectopic calcifications in beta-Thalassemia patients
QUAGLINO, Daniela;BORALDI, Federica;ANNOVI, Giulia;
2013
Abstract
A surprisingly high percentage of clinical complications affecting beta-thalassemia (beta-thal) patients is due to ectopic calcification. In these patients, elastic fiber calcification is associated with accumulation of anion superoxide and to increased levels of oxidized proteins and lipids. As a consequence, carboxylation of the calcification inhibitor Matrix Gla Protein (MGP) might be impaired. Independently from the gene defect, common pathogenic pathways are associated with ectopic calcification in PXE and in a number of beta-thal patients.Pubblicazioni consigliate
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