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

Lim, Wai Feng; Muniandi, Logeswaran; George, Elizabeth; Sathar, Jameela; Teh, Lai Kuan; Gan, Gin Gin; Lai, Mei I

doi:10.1016/j.bcmd.2011.10.002

Cited by 12 publications

(19 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Lim et al [7] suggested that AHSP is a secondary compensatory mechanism, in addition to the HbF response, to the α/β globin chain imbalance in HbE/beta thalassemia patients. HPLC analysis in this group of patients revealed elevated HbF, ranging from 5-47%, in 48% of the patients (Fig.…”

Section: Hbf and Ahsp Levelmentioning

confidence: 99%

“…These phenotypes could be the result of the loss of AHSP, causing nascent α-globin to be structurally unstable, making it incompatible for HbA formation [7]. Lim et al [7] have reported significant correlation between AHSP expression and mean cell hemoglobin, HbF %, α-globin, β-globin and excess α-globin expression and conclude that AHSP could be a secondary compensatory mechanism in red blood cells to counterbalance the excess α-globin chains in HbE/β-thalassemia individuals.…”

Section: Introductionmentioning

confidence: 97%

See 1 more Smart Citation

Alpha hemoglobin stabilizing protein: Its causal relationship with the severity of beta thalassemia

Sagar

Kumar

Sharma

et al. 2015

Blood Cells, Molecules, and Diseases

View full text Add to dashboard Cite

Section: Hbf and Ahsp Levelmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 97%

Alpha hemoglobin stabilizing protein: Its causal relationship with the severity of beta thalassemia

Sagar

Kumar

Sharma

et al. 2015

Blood Cells, Molecules, and Diseases

View full text Add to dashboard Cite

“…Severe patients, compared with mild patients, had an increase in markers of RBC oxidative stress based on ineffective erythropoiesis, lower RBC counts, PS exposure, greater serum EPO and plasma malondialdehyde (MDA), and RBC SOD. Other laboratories have shown not only an increase in RBC redox enzymes, such as PRDX2, catalase, thioredoxin, and SOD, but also an increase in the alpha globin chaperone, alpha hemoglobinstabilizing protein, negatively correlated with MCH and HbF (18,102,189).…”

Section: Fig 2 Hbe Acts As a Bmentioning

confidence: 99%

“…With the unraveling of complex genetic and environmental factors modulating clinical severity, new potential therapeutic approaches are under development [e.g., (65,147)]: gene switching (19) that includes HbF induction by hydroxyurea; stem cell transplants; gene therapy; pharmacological targeting of defective erythropoiesis; targeting free a-chains and with a-Hb chaperones; and countering the vasculature depletion of NO from chronic hemolysis (18,19,36,102,111,133,179,189).…”

Section: Current Therapeutics Addressing Oxidative Stress and Iron Ovmentioning

confidence: 99%

HbE/β-Thalassemia and Oxidative Stress: The Key to Pathophysiological Mechanisms and Novel Therapeutics

Hirsch

Sibmooh

Fucharoen

et al. 2017

Antioxidants & Redox Signaling

View full text Add to dashboard Cite

Significance: Oxidative stress and generation of free radicals are fundamental in initiating pathophysiological mechanisms leading to an inflammatory cascade resulting in high rates of morbidity and death from many inherited point mutation-derived hemoglobinopathies. Hemoglobin (Hb)E is the most common point mutation worldwide. The b E -globin gene is found in greatest frequency in Southeast Asia, including Thailand, Malaysia, Indonesia, Vietnam, Cambodia, and Laos. With the wave of worldwide migration, it is entering the gene pool of diverse populations with greater consequences than expected. Critical Issues: While HbE by itself presents as a mild anemia and a single gene for b-thalassemia is not serious, it remains unexplained why HbE/b-thalassemia (HbE/b-thal) is a grave disease with high morbidity and mortality. Patients often exhibit defective physical development, severe chronic anemia, and often die of cardiovascular disease and severe infections. Recent Advances: This article presents an overview of HbE/b-thal disease with an emphasis on new findings pointing to pathophysiological mechanisms derived from and initiated by the dysfunctional property of HbE as a reduced nitrite reductase concomitant with excess a-chains exacerbating unstable HbE, leading to a combination of nitric oxide imbalance, oxidative stress, and proinflammatory events. Future Directions: Additionally, we present new therapeutic strategies that are based on the emerging molecular-level understanding of the pathophysiology of this and other hemoglobinopathies. These strategies are designed to short-circuit the inflammatory cascade leading to devastating chronic morbidity and fatal consequences. Antioxid. Redox Signal. 26,[794][795][796][797][798][799][800][801][802][803][804][805][806][807][808][809][810][811][812][813]

show abstract

“…Persons with HbE/β-thalassemia, AHSP can compensate the toxic effects of excess α-globin chains in RBCs [46]. However, some studies have shown that AHSP was not adequately associated with severity of β-thalassemia as a genetic modifier [47].…”

Section: Alpha-hb-stabilizing Proteinmentioning

confidence: 98%

Review: Beta-thalassemia and molecular chaperones

Sumera

Radhakrishnan

Baba

et al. 2015

Blood Cells, Molecules, and Diseases

Self Cite

View full text Add to dashboard Cite

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

Cited by 12 publications

References 18 publications

Alpha hemoglobin stabilizing protein: Its causal relationship with the severity of beta thalassemia

Alpha hemoglobin stabilizing protein: Its causal relationship with the severity of beta thalassemia

HbE/β-Thalassemia and Oxidative Stress: The Key to Pathophysiological Mechanisms and Novel Therapeutics

Review: Beta-thalassemia and molecular chaperones

Contact Info

Product

Resources

About