Determination of alpha hemoglobin stabilizing protein (AHSP) gene expression and oxidative stress parameters in HbE/beta-Thalassemia

Beta-Thalassaemia is the genetic disorders of haemoglobin synthesis characterized by reduced or absent β-globin chain synthesis thus lead to imbalance of tetramer. The Alpha Haemoglobin Stabilizing Protein (AHSP) acts as a molecular chaperone for α-globin by stabilizing free α-globin preventing it f...

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Main Author: Yaacob, Nur Suraya Che
Format: Thesis
Language:English
Published: 2021
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Online Access:http://eprints.usm.my/50464/1/NUR%20SURAYA%20BINTI%20CHE%20YAACOB-%20FINAL%20THESIS%20P-UM000819%28R%29-24%20pages.pdf
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spelling my.usm.eprints.50464 http://eprints.usm.my/50464/ Determination of alpha hemoglobin stabilizing protein (AHSP) gene expression and oxidative stress parameters in HbE/beta-Thalassemia Yaacob, Nur Suraya Che RK Dentistry Beta-Thalassaemia is the genetic disorders of haemoglobin synthesis characterized by reduced or absent β-globin chain synthesis thus lead to imbalance of tetramer. The Alpha Haemoglobin Stabilizing Protein (AHSP) acts as a molecular chaperone for α-globin by stabilizing free α-globin preventing it from precipitating and forming reactive oxygen species byproducts with subsequent oxidative damage and the formation of intracellular precipitates thus led to oxidative stress. Real time PCR and ELISA assay were used to determine the AHSP expression and oxidative stress parameters (SOD activity and MDA concentration) in blood sample respectively. Expression of AHSP significant in disease severity (p=0.001), transfusion dependency (p=0.033), molecular profile (p=0.035) and age (p=0.034), SOD activity significant in disease severity (p=0.005), transfusion dependency (p=0.001), serum ferritin level (p=0.005) and age (p=0.040), and MDA concentration significant in disease severity (p=0.003), transfusion dependency (p=0.001), splenectomy status (p=0.002), serum ferritin level (p=0.002) and age (p=0.015). We found that AHSP expression was significantly correlated to HbF (p=0.033) and frequency of blood transfusion per year (0.011) while SOD activity significantly correlated with age (p=0.033), HbF (p=0.009) and frequency of blood transfusion per year (p=0.004). On the other hand, MDA concentration was significantly correlated with age (p=0.008) and serum ferritin (p=0.022). In addition, correlation study was evaluated between AHSP expression with SOD activity and MDA concentration in which AHSP expression was significantly correlated with both parameters when p=0.002 and p=0.001 respectively. Thus, we concluded that AHSP could be a secondary compensatory mechanism in red blood cells to counterbalance the excess α-globin chains thus reduced the oxidative stress in HbE/Beta-Thalassaemia individuals. AHSP modifier and oxidative stress parameters give the future insight on its role in disease pathogenesis. 2021-07 Thesis NonPeerReviewed application/pdf en http://eprints.usm.my/50464/1/NUR%20SURAYA%20BINTI%20CHE%20YAACOB-%20FINAL%20THESIS%20P-UM000819%28R%29-24%20pages.pdf Yaacob, Nur Suraya Che (2021) Determination of alpha hemoglobin stabilizing protein (AHSP) gene expression and oxidative stress parameters in HbE/beta-Thalassemia. Masters thesis, Universiti Sains Malaysia.
institution Universiti Sains Malaysia
building Hamzah Sendut Library
collection Institutional Repository
continent Asia
country Malaysia
content_provider Universiti Sains Malaysia
content_source USM Institutional Repository
url_provider http://eprints.usm.my/
language English
topic RK Dentistry
spellingShingle RK Dentistry
Yaacob, Nur Suraya Che
Determination of alpha hemoglobin stabilizing protein (AHSP) gene expression and oxidative stress parameters in HbE/beta-Thalassemia
description Beta-Thalassaemia is the genetic disorders of haemoglobin synthesis characterized by reduced or absent β-globin chain synthesis thus lead to imbalance of tetramer. The Alpha Haemoglobin Stabilizing Protein (AHSP) acts as a molecular chaperone for α-globin by stabilizing free α-globin preventing it from precipitating and forming reactive oxygen species byproducts with subsequent oxidative damage and the formation of intracellular precipitates thus led to oxidative stress. Real time PCR and ELISA assay were used to determine the AHSP expression and oxidative stress parameters (SOD activity and MDA concentration) in blood sample respectively. Expression of AHSP significant in disease severity (p=0.001), transfusion dependency (p=0.033), molecular profile (p=0.035) and age (p=0.034), SOD activity significant in disease severity (p=0.005), transfusion dependency (p=0.001), serum ferritin level (p=0.005) and age (p=0.040), and MDA concentration significant in disease severity (p=0.003), transfusion dependency (p=0.001), splenectomy status (p=0.002), serum ferritin level (p=0.002) and age (p=0.015). We found that AHSP expression was significantly correlated to HbF (p=0.033) and frequency of blood transfusion per year (0.011) while SOD activity significantly correlated with age (p=0.033), HbF (p=0.009) and frequency of blood transfusion per year (p=0.004). On the other hand, MDA concentration was significantly correlated with age (p=0.008) and serum ferritin (p=0.022). In addition, correlation study was evaluated between AHSP expression with SOD activity and MDA concentration in which AHSP expression was significantly correlated with both parameters when p=0.002 and p=0.001 respectively. Thus, we concluded that AHSP could be a secondary compensatory mechanism in red blood cells to counterbalance the excess α-globin chains thus reduced the oxidative stress in HbE/Beta-Thalassaemia individuals. AHSP modifier and oxidative stress parameters give the future insight on its role in disease pathogenesis.
format Thesis
author Yaacob, Nur Suraya Che
author_facet Yaacob, Nur Suraya Che
author_sort Yaacob, Nur Suraya Che
title Determination of alpha hemoglobin stabilizing protein (AHSP) gene expression and oxidative stress parameters in HbE/beta-Thalassemia
title_short Determination of alpha hemoglobin stabilizing protein (AHSP) gene expression and oxidative stress parameters in HbE/beta-Thalassemia
title_full Determination of alpha hemoglobin stabilizing protein (AHSP) gene expression and oxidative stress parameters in HbE/beta-Thalassemia
title_fullStr Determination of alpha hemoglobin stabilizing protein (AHSP) gene expression and oxidative stress parameters in HbE/beta-Thalassemia
title_full_unstemmed Determination of alpha hemoglobin stabilizing protein (AHSP) gene expression and oxidative stress parameters in HbE/beta-Thalassemia
title_sort determination of alpha hemoglobin stabilizing protein (ahsp) gene expression and oxidative stress parameters in hbe/beta-thalassemia
publishDate 2021
url http://eprints.usm.my/50464/1/NUR%20SURAYA%20BINTI%20CHE%20YAACOB-%20FINAL%20THESIS%20P-UM000819%28R%29-24%20pages.pdf
http://eprints.usm.my/50464/
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score 13.211869