Dr. Zafar H. Zaidi Center for Proteomics, University of Karachi, Karachi, Pakistan.
Omair Sana Foundation, Karachi, Pakistan.
Fetal Pediatr Pathol. 2021 Dec;40(6):563-570. doi: 10.1080/15513815.2020.1725940. Epub 2020 Feb 16.
β-thalassemia is a genetic disorder characterized by reduction or absence of β-globin chain with mutations in both copies (β-thalassemia major) or in one copy (β-thalassemia minor). Pregnancies in β- thalassemic carrier women are considered symptom free but have risk of inheriting β-thalassemic fetuses. Current study was designed to compare oxidative stress and antioxidants status in maternal serum from β-thalassemic minor mothers having β-thalassemic major and normal fetuses. We investigated paraoxonase (PON1) and arylesterase (ARE) activities along with malondialdehyde (MDA) and reactive oxygen species (ROS) in maternal serum of β-thalassemic carrier women. PON1 and ARE activities were found to be significantly decreased, whereas the concentration of MDA and ROS were significantly increased in β-thalassemic minor mothers with β-thalassemic major fetuses. The study concludes that redox imbalance in β-thalassemic trait mothers carrying thalassemic fetuses is higher than in mothers carrying normal fetuses.
β-地中海贫血是一种遗传性疾病,其特征是β-珠蛋白链减少或缺失,且两条染色体(β-地中海贫血重型)或一条染色体(β-地中海贫血轻型)均发生突变。β-地中海贫血携带者的妊娠通常无症状,但有遗传β-地中海贫血胎儿的风险。本研究旨在比较携带重型β-地中海贫血和正常胎儿的β-地中海贫血轻型母亲的母血清中的氧化应激和抗氧化剂状态。我们研究了母体血清中对氧磷酶 1(PON1)和芳基酯酶(ARE)的活性以及丙二醛(MDA)和活性氧(ROS)的浓度。结果发现,携带重型β-地中海贫血胎儿的β-地中海贫血轻型母亲的 PON1 和 ARE 活性显著降低,而 MDA 和 ROS 的浓度显著升高。研究结论是,携带地中海贫血胎儿的β-地中海贫血表型母亲的氧化还原失衡高于携带正常胎儿的母亲。