title: α-Haemoglobin stabilising protein expression is influenced by mean cell haemoglobin and HbF levels in HbE/β-thalassaemia individuals.
abstract: The alpha haemoglobin stabilising protein (AHSP) acts as a molecular chaperone for α-globin by stabilising nascent α-globin before transferring it to waiting free β-globin chains. Binding of AHSP to α-globin renders α-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 β-thalassaemia. Studies have shown that AHSP is a modifier in β-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 β-thalassaemia, whereas others have shown that certain AHSP haplotype could modify the phenotype of β-thalassaemia intermedia patients. We investigated the expression of AHSP in relation to selected demographic data, full blood count, HPLC results, HbE/β-thalassaemia genotype, Xmn-1 Gγ polymorphism, α-globin, β-globin and γ-globin expression. We found that AHSP expression was significantly correlated to mean cell haemoglobin level, HbF %, α-globin, β-globin and excess α-globin expression. We concluded that AHSP could be a secondary compensatory mechanism in red blood cells to counterbalance the excess α-globin chains in HbE/β-thalassaemia individuals.
authors.affiliation: Department of Pathology, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, Malaysia.
authors.forename: Wai Feng
authors.initials: WF
authors.lastname: Lim
authors.forename: Logeswaran
authors.initials: L
authors.lastname: Muniandi
authors.forename: Elizabeth
authors.initials: E
authors.lastname: George
authors.forename: Jameela
authors.initials: J
authors.lastname: Sathar
authors.forename: Lai Kuan
authors.initials: LK
authors.lastname: Teh
authors.forename: Gin Gin
authors.initials: GG
authors.lastname: Gan
authors.forename: Mei I
authors.initials: MI
authors.lastname: Lai
mesh_terms: D000293:Adolescent; D000328:Adult; D000818:Animals; D044466:Asian People / Q000235:genetics*; D001798:Blood Proteins / Q000235:genetics*; D004909:Erythrocyte Indices / Q000235:genetics; D004912:Erythrocytes; D005260:Female; D005319:Fetal Hemoglobin / Q000235:genetics*; D015870:Gene Expression; D006239:Haplotypes; D006446:Hemoglobin E / Q000235:genetics*; D006801:Humans; D008297:Male; D051379:Mice; D008875:Middle Aged; D018832:Molecular Chaperones / Q000235:genetics*; D010641:Phenotype; D055542:alpha-Globins / Q000235:genetics*; D055544:beta-Globins / Q000235:genetics; D017086:beta-Thalassemia / Q000235:genetics*; D055545:gamma-Globins / Q000235:genetics
chemical_list: C468768:AHSP protein, human; D001798:Blood Proteins; D018832:Molecular Chaperones; D055542:alpha-Globins; D055544:beta-Globins; D055545:gamma-Globins; D006446:Hemoglobin E; D005319:Fetal Hemoglobin