title

اثرات دفریپرون و دفراسیروکس بر ساختار و عملکرد هموگلوبین بتا تالاسمی

موسوی موحدی, علی اکبر ، موسوی, سیدجعفر ، دیوسالار, عادله ، بابا احمدی, ابوذر ، کریمیان, خشایار ، شفیعی, عباس ، کمره ای, محمود ، پورساسان, نغمه ، فرزامی, بیژن ، ریاضی, غلام حسین ، حکیم الهی, غلامحسین ، تسای, فو یوان ، احمد, فیضان ، امانی, مجتبی ، صبوری, علی اکبر (1388) اثرات دفریپرون و دفراسیروکس بر ساختار و عملکرد هموگلوبین بتا تالاسمی. Journal of Biomolecular Structure and Dynamics ــ 27 (3). ص.ص.319-329. شاپا 0739-1102

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عنوان انگليسی

The effects of deferiprone and deferasirox on the structure and function of β-thalassemia hemoglobin

خلاصه انگلیسی

Transfusional iron overload is a major cause of morbidity and mortality in thalassemia, sickle-cell disease and other chronic anemias. To overcome these problems, orally bio available iron chelators, deferiprone and deferasirox, were used for the treatment of patients suffering from thalassemia. The interactions between deferiprone and deferasirox with the carrier protein, β-thalassemia hemoglobin (Hb), were investigated using fluorescence, circular dichroism (CD) and UV-visible measurements at physiological condition. Strong fluorescence quenching on interactions of the above drugs with β-thalassemia Hb were observed. Fluorescence quenching data of thalassemia Hb in the presence of deferasirox have shown greater affinity of binding. The number of binding sites to Hb for deferasirox was found to be more relative to those of the deferiprone. The effects of these drugs on the oxygen affinity of the thalassemia Hb were studied by spectroscopic methods using sodium dithionite. Results indicated that deferiprone reduces oxygen affinity (increases oxygen releasing ability) of Hb, while in the presence of deferasirox, oxygen affinity of Hb has significantly increased by dose-dependent manner. As such, deferasirox exhibited opposite effect relative to deferiprone on the function of thalassemia Hb. In clinical dose of deferiprone, CD results showed that, the α-helical content of thalassemia Hb significantly increased. By use of the clinical dose of deferasirox, however, a decrease in α-helical content of protein was observed, which resulted in decreasing stability of thalassemia Hb. Our study showed that reduction in stability of thalassemia Hb in the presence of deferasirox induced higher conformational changes in protein. ©Adenine Press (2009).

نوع سند :مقاله
زبان سند : انگلیسی
نویسنده مسئول :علی اکبر موسوی موحدی
ضریب تاثیر و نمایه مجلات:cited By (since 1996) 0 1- Indexing: MEDLINE/PubMed 2- Scopus
کلیدواژه ها (انگلیسی):beta thalassemia hemoglobin; deferasirox; deferiprone; hemoglobin; sodium dithionite; unclassified drug, article; beta thalassemia; binding affinity; circular dichroism; drug effect; fluorescence; human; human cell; oxygen affinity; priority journal; protein stability; ultraviolet radiation, Benzoic Acids; beta-Thalassemia; Circular Dichroism; Hemoglobins; Humans; Iron Chelating Agents; Pyridones; Spectrometry, Fluorescence; Structure-Activity Relationship; Triazoles
موضوعات :QU بیوشیمی
بخش های دانشگاهی :دانشكده پزشكي > گروه علوم پایه > بخش بیوفیزیک
کد شناسایی :1830
ارائه شده توسط : دکتر مجتبی امانی
ارائه شده در تاریخ :08 آذر 1389 01:47
آخرین تغییر :20 فروردین 1393 12:47

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