alpha-Haemoglobin stabilising protein expression is influenced by mean cell haemoglobin and HbF levels in HbE/beta-thalassaemia individuals

作者：Lim Wai Feng; Muniandi Logeswaran; George Elizabeth; Sathar Jameela; Teh Lai Kuan; Gan Gin Gin; Lai Mei I^*

来源：Blood Cells, Molecules, and Diseases, 2012, 48(1): 17-21.

DOI：10.1016/j.bcmd.2011.10.002

• AHSP
• HbE/beta-thalassaemia
• MCV
• MCH
• HbF

The alpha haemoglobin stabilising protein (AHSP) acts as a molecular chaperone for alpha a-globin by stabilising nascent alpha-globin before transferring it to waiting free beta-globin chains. Binding of AHSP to alpha-globin renders alpha-globin chemically inert whereby preventing it from precipitating and forming reactive oxygen species byproducts. The AHSP has been actively studied in the recent years, particularly in its relation to beta-thalassaemia. Studies have shown that AHSP is a modifier in beta-thalassaemia mice models. However, this relationship is less established in humans. Studies by some groups showed no correlation between the AHSP haplotypes and the severity of beta-thalassaemia, whereas others have shown that certain AHSP haplotype could modify the phenotype of beta-thalassaemia intermedia patients. We investigated the expression of AHSP in relation to selected demographic data, full blood count, HPLC results, HbE/beta-thalassaemia genotype, Xmn-1 G gamma polymorphism, alpha-globin, beta-globin and gamma-globin expression. We found that AHSP expression was significantly correlated to mean cell haemoglobin level, HbF %, alpha-globin, beta-globin and excess alpha-globin expression. We concluded that AHSP could be a secondary compensatory mechanism in red blood cells to counterbalance the excess a-globin chains in HbE/beta-thalassaemia individuals.

• 出版日期2012-1-15