Isolated erythrocyte membranes of transfusion‐dependent and non‐transfused thalassemia patients in Jakarta, investigated by electron paramagnetic resonance spectroscopy

Udyaningsih-Freisleben, Seruni K.; Kurniati, V.; Prasetyo, Pamulih B.; Handayani, Sri; Adhiyanto, Chris; Soegianto, Rondang R.; Pudiantari, Ratna; Munthe, Bulan Ginting; Ramelan, Wahyuning; Freisleben, Hans-Joachim

doi:10.1002/biof.5520190111

Cited by 6 publications

(8 citation statements)

References 30 publications

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“…Vice versa, parameters of lipid peroxidation increase, measured as TBARs, e.g., MDA, a parameter of membrane damage. This is confirmed by the investigation of isolated red cell membranes by ESR spectroscopy and SDS-PAGE [27].…”

Section: Discussionsupporting

confidence: 57%

Iron status and oxidative stress in β‐thalassemia patients in Jakarta

Laksmitawati

Handayani

Udyaningsih-Freisleben

et al. 2003

BioFactors

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Abstract.A study on thalassemia intermedia and major patients in Jakarta was initiated to obtain a comprehensive picture of metabolic dysregulation, iron overload, oxidative stress, and cell damage. Data are presented from a group of 14 transfusiondependent patients in an age range of 11-25 years (T) and another group of 9 frequently transfused (for at least 15 years) patients aged 17-30 years (L). A third group comprised 6 patients (aged 7 to 14 years) who had not yet obtained transfusions (N). The 21 controls (C) were voluntary students without diagnosis or clinical signs of thalassemia up to 30 years of age. The study was approved by the Ethical Clearance Board of the Medical Faculty and all blood samples from controls and patients were obtained on fully informed consent. Levels of antioxidants (vitamins A, C, E and β-carotene) and reactive thiols are considerably decreased in transfused patients, whereas signs of iron overload and cell damage are increased (serum iron, ferritin, transferrin saturation, SGOT, SGPT, γ-GT, bilirubin). Results can be summarized that non-transfused thalassemia intermedia patients exert slight signs of oxidative stress, and increased hemoglobin degradation but no significant indication of tissue or cell damage. This picture differs considerably from transfusion-dependent thalassemia major patients: highly significant decrease in antioxidants and thiols and tremendous iron overload and cell damage. The picture is even worsened in long-term transfused patients. Iron chelation after transfusion is not sufficient in Indonesia, because it is normally (with few exceptions) applied only once together with transfusion. Hence, one major reason of the bad condition of transfusion-dependent thalassemia patients in Indonesia appears to be frequent transfusions (on the average one per month) and insufficient chelation of one treatment per month together with transfusion.

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Section: Discussionsupporting

confidence: 57%

Iron status and oxidative stress in β‐thalassemia patients in Jakarta

Laksmitawati

Handayani

Udyaningsih-Freisleben

et al. 2003

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“…5 In the frame of our previous studies, we reported on hematology, iron overload, imbalanced antioxidant status 6 and changes in characteristics of isolated RBC membranes in thalassemic patients in Jakarta. 7 Now, we compare the plasma lipid pattern under oxidative stress with parameters of isolated RBC membranes from thalassemic patients measured with electron paramagnetic resonance spectroscopy using doxyl-stearic acid spin labels and thiol-reactive N-proxyl-maleimide spin label.…”

mentioning

confidence: 99%

Plasma lipid pattern and red cell membrane structure in β-thalassemia patients in Jakarta

Freisleben¹,

Hidayat²,

Freisleben³

et al. 2011

Med J Indones

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Latar belakang: Kami melakukan penelitian selama 10 tahun terakhir untuk mendapatkan gambaran yang komprehensif mengenai kelebihan besi, stres oksidatif, dan kerusakan sel pada pasien talasemia di Jakarta. Metode: Dari sampel darah 15 pasien yang bergantung transfusi (kelompok T), 5 pasien yang belum ditransfusi (kelompok N) dan 10 kontrol (kelompok C) dilakukan analisis plasma dan membran eritrosit yang diisolasi dan strukturnya diteliti dengan spekroskopi electron paramagnetic resonance (EPR) menggunakan doxyl-stearic acid dan maleimido-proxyl spin lable. Data statistik dianalisis dengan ANOVA. Hasil: Terdapat nilai trigliserida lebih tinggi dan nilai kolesterol plasma lebih rendah pada penderita talasemia dibandingkan dengan kontrol. Kadar vitamin E (kelompok C: 21.8 vs T: 6.2 μmol/L) dan reactive thiols (C: 144 vs. T: 61 μmol/L) jauh lebih rendah pada pasien yang bergantung-transfusi. Tanda-tanda stres oksidatif (MDA, C: 1.96 vs. T: 9.2 μmol/L) dan kadar transaminase dalam serum sangat tinggi. Pasien belum ditransfusi memperlihatkan sedikit tanda-tanda terjadinya stres oksidatif, tetapi tidak terdapat tanda-tanda yang berarti adanya kerusakan jaringan atau sel. Parameter membran sel darah merah dari spektroskopi electron paramagnetic resonannce (EPR) menunjukkan hasil yang sangat berbeda-beda untuk setiap kelompok. Pada pasien bergantung-transfusi, struktur membran sel darah merah serta gradien polaritas dan fl uiditas dalam domain lipid menunjukkan rusak; kapasitas mengikat protein tiol di dalam membran lebih rendah dan statis.

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“…Free iron can generate reactive oxygen species (ROS), which cause damage to tissues, especially to the liver and the heart (8)(9)(10). Excess iron in the body must be treated with iron chelators (11,12), e.g., deferoxamin (DFO) as one of the standard drugs.…”

Section: Estuningtyas Et Almentioning

confidence: 99%