Ranjbaran Reza, Okhovat Mohammad Ali, Mobarhanfard Arash, Aboualizadeh Farzaneh, Abbasi Mozhdeh, Moezzi Leili, Golafshan Habib Allah, Behzad-Behbahani Abbas, Bagheri Mandana, Sharifzadeh Sedigheh
PhD.; Deputy Paramedical Education, School of Paramedical Sciences, Shiraz University of Medical Sciences, Shiraz, Iran; phone:+987112270240;
Ann Clin Lab Sci. 2014 Spring;44(2):189-93.
Alpha hemoglobin stabilizing protein (AHSP) is a chaperone-like molecule specialized for erythroid series which binds to free α-globin chain. According to this characteristic, AHSP can be considered an important factor which reduces beta thalassemia symptoms.
Reticulocytes RNA extraction and a subsequent cDNA synthesis were performed, followed by Relative qRT-PCR for AHSP, alpha, and beta globin chain genes. The beta actin gene was used as an endogenous reference as well. The relationship between AHSP gene expression, disease severity, and the β/α globin mRNA ratio was studied among different homozygote β-thalassemia patients (mild, moderate and severe) and compared with minor thalassemia and the normal population.
Analysis of the β-globin/α-globin mRNA ratio has shown that disease severity enhanced with a decrease in this proportion. Evaluation of the correlation between AHSP gene expression and the average of the β-globin/α-globin expression ratio indicated a significant but indirect relationship in considered groups. Our results demonstrated that the AHSP gene expression increases in accordance with augmentation of clinical symptoms.
Although one of the main reasons for reduced clinical severity in homozygote β-thalassemia can be the high level of AHSP gene expression as a chaperon molecule, our findings indicated that AHSP gene expression decreased in a mild category as compared to that in severe and moderate groups.
α血红蛋白稳定蛋白（AHSP）是一种专门作用于红系的伴侣样分子，可与游离的α珠蛋白链结合。基于这一特性，AHSP可被视为减轻β地中海贫血症状的重要因素。
进行网织红细胞RNA提取及后续的cDNA合成，然后对AHSP、α和β珠蛋白链基因进行相对定量逆转录聚合酶链反应（Relative qRT-PCR）。β肌动蛋白基因也用作内参。研究不同纯合子β地中海贫血患者（轻度、中度和重度）中AHSP基因表达、疾病严重程度与β/α珠蛋白mRNA比值之间的关系，并与轻型地中海贫血患者和正常人群进行比较。
β珠蛋白/α珠蛋白mRNA比值分析表明，该比例降低时疾病严重程度增加。对AHSP基因表达与β珠蛋白/α珠蛋白表达比值平均值之间的相关性评估表明，在所研究的组中存在显著但间接的关系。我们的结果表明，AHSP基因表达随临床症状的加重而增加。
虽然纯合子β地中海贫血临床严重程度降低的主要原因之一可能是作为伴侣分子的AHSP基因表达水平较高，但我们的研究结果表明，与重度和中度组相比，轻度组中AHSP基因表达降低。
