title: Comparative plasma protein profiling of hemoglobin H disease.
abstract: HbH and HbH-constant spring (HbH-CS) are the most common forms of α-thalassemia detected in the Thai population. The accumulation of excess β globin chains in these diseases results in increased red cell hemolysis, and patients with HbH-CS normally have a more severe clinical presentation than patients with HbH disease. This study aimed to detect alterations in the expression of plasma proteins of HbH and HbH-CS patients as compared to normal plasma. Platelet poor plasma was separated from HbH and HbH-CS and normal subjects and differential plasma proteins were detected using two-dimensional gel electrophoresis and identified using LC/MS/MS. A total of 14 differentially expressed proteins were detected of which 5 proteins were upregulated and 9 were downregulated. Most of the differentially expressed proteins are liver secreted proteins involved in hemolysis, oxidative stress response, and hemoglobin degradation. Seven proteins were found to be differentially expressed between HbH and HbH-CS. Levels of haptoglobin, a hemoglobin scavenging protein, were significantly increased in HbH patients as compared to HbH-CS patients. The identification of differentially expressed proteins may lead to a better understanding of the biological events underlying the clinical presentation of HbH and HbH-CS patients and can have application as hemolytic markers or severity predictors.
authors.affiliation: Department of Clinical Microscopy, Faculty of Allied Health Sciences, Chulalongkorn University, 254 Phayathai Road, Bangkok 10330, Thailand.
authors.forename: Kamonlak
authors.identifier: 0000-0003-2032-1832
authors.initials: K
authors.lastname: Leecharoenkiat
authors.affiliation: Molecular Pathology Laboratory, Institute of Molecular Biosciences, Mahidol University, 25/25 Phuttamonthon 4 Road, Salaya, Nakhon Pathom 73170, Thailand.
authors.forename: Wannapa
authors.identifier: 0000-0003-0935-7930
authors.initials: W
authors.lastname: Sornjai
authors.affiliation: Molecular Pathology Laboratory, Institute of Molecular Biosciences, Mahidol University, 25/25 Phuttamonthon 4 Road, Salaya, Nakhon Pathom 73170, Thailand.
authors.forename: Kornpat
authors.initials: K
authors.lastname: Khungwanmaythawee
authors.affiliation: Proteomics Research Laboratory, Genome Institute, National Science and Technology Development Agency, 111 Thailand Science Park, Phahonyothin Road, Khlong Nueng, Khlong Luang, Pathum Thani 12120, Thailand.
authors.forename: Atchara
authors.initials: A
authors.lastname: Paemanee
authors.affiliation: Proteomics Research Laboratory, Genome Institute, National Science and Technology Development Agency, 111 Thailand Science Park, Phahonyothin Road, Khlong Nueng, Khlong Luang, Pathum Thani 12120, Thailand.
authors.forename: Chartchai
authors.initials: C
authors.lastname: Chaichana
authors.affiliation: Proteomics Research Laboratory, Genome Institute, National Science and Technology Development Agency, 111 Thailand Science Park, Phahonyothin Road, Khlong Nueng, Khlong Luang, Pathum Thani 12120, Thailand.
authors.forename: Sittiruk
authors.initials: S
authors.lastname: Roytrakul
authors.affiliation: Thalassemia Research Center, Institute of Molecular Biosciences, Mahidol University, 25/25 Phuttamonthon 4 Road, Salaya, Nakhon Pathom 73170, Thailand.
authors.forename: Suthat
authors.initials: S
authors.lastname: Fucharoen
authors.affiliation: Thalassemia Research Center, Institute of Molecular Biosciences, Mahidol University, 25/25 Phuttamonthon 4 Road, Salaya, Nakhon Pathom 73170, Thailand.
authors.forename: Saovaros
authors.initials: S
authors.lastname: Svasti
authors.affiliation: Molecular Pathology Laboratory, Institute of Molecular Biosciences, Mahidol University, 25/25 Phuttamonthon 4 Road, Salaya, Nakhon Pathom 73170, Thailand.
authors.forename: Duncan R
authors.initials: DR
authors.lastname: Smith
mesh_terms: D016022:Case-Control Studies; D005260:Female; D006242:Haptoglobins / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D008297:Male; D017085:alpha-Thalassemia / Q000097:blood*
chemical_list: D006242:Haptoglobins