مقالات پذیرفته شده در نهمین کنگره بین المللی زیست پزشکی
Common Polymorphisms Associated with Beta-Thalassemia Minor
Common Polymorphisms Associated with Beta-Thalassemia Minor
Dr. Majid MesgarTehrani,1,*Fatima yarahmadi,2Dr. Mohammad Mahdi Eslami,3Dr. Reza Mirlohi,4
1. Member of the Core Committee of the National Genomics Hub, Shahid Beheshti University of Medical Sciences, Tehran, Iran 3. Member of the Bioinformatics Research Group, Nasim Research Institute, Tehran, Iran 4. Member of the Bioinformatics Research Group, Nasim Research Institute, Tehran, Iran
Introduction: Minor thalassemia is one of the most common genetic blood disorders
worldwide, particularly prevalent in the Mediterranean, Middle Eastern,
South Asian, and North African regions. This disease results from a
reduction or defect in the production of _β globin chains, leading to
abnormalities in the structure of hemoglobin. Due to the high genetic
polymorphism diversity involved in this condition, a wide spectrum of
clinical symptoms is observed, ranging from mild anemia to more
severe manifestations. Therefore, identifying and studying the genetic
polymorphisms associated with minor thalassemia is of great
importance for diagnosis, disease prognosis, and the development of
effective treatments.
Recent studies published in the past five years have shown that
polymorphisms in the α- and β-globin genes, as well as their regulatory
regions, play a key role in determining disease severity and the
production level of fetal hemoglobin (HbF). Increased levels of HbF are
recognized as a protective factor that can alleviate the effects of anemia
and mitigate disease progression. A study conducted in 2022
demonstrated that polymorphisms located in the BCL11A and HBS1L-
MYB regions are directly associated with increased HbF levels in
patients with minor thalassemia and can prevent the occurrence of
severe symptoms.
In addition, numerous genetic studies have shown that certain
polymorphisms in genes regulating iron metabolism, such as HbF and
TMPRSS6, can influence iron accumulation in patients with minor
thalassemia and increase the risk of complications related to iron
overload, such as liver fibrosis and cardiac tissue damage. These
findings highlight the importance of genetic screening after the
initiation of supportive treatments, especially frequent blood
transfusions, and pave the way for the development of personalized
therapies.
Other studies have shown that genetic polymorphisms may influence
the response of patients with minor thalassemia to pharmacological
treatments such as hydroxyurea. Hydroxyurea is known as a fetal
hemoglobin (HbF) inducer; however, its effectiveness can vary among
individuals, and these differences are largely attributed to genetic
polymorphisms.
In addition, population-based genetic differences also play a significant
role in the prevalence and types of polymorphisms. Studies conducted
in various populations have shown that certain polymorphisms are
more common in specific geographic regions, and these genetic
variations should be considered in the design of preventive and
therapeutic strategies. For instance, certain polymorphisms are more
prevalent in Middle Eastern and Asian populations compared to
European populations, and vice versa.
Global Status of Minor Thalassemia
•Global prevalence: In 2021, the age-standardized prevalence rate of
thalassemia worldwide was 18.28 cases per 100,000 population.
•Global mortality: The age-standardized mortality rate in 2021 was
reported as 0.15 deaths per 100,000 population.
•Gender differences: In most age groups, the prevalence is higher in
males than in females.
Regional and Country-Specific Status
•Middle East and South Asia: Countries such as Iran, Iraq, and India
have the highest prevalence rates of minor thalassemia.
•Europe: Mediterranean countries like Greece and Italy exhibit higher
prevalence of this disease.
•United States: In the U.S., thalassemia-related mortality rates vary
among racial groups, with Asians having the highest mortality rate.
Given the heavy burden of thalassemia in many countries, especially
Iran, which is one of the regions with a high prevalence of thalassemia
minor, precise identification of polymorphisms and a thorough
understanding of the related molecular mechanisms can be effective in
improving diagnostic methods, better predicting disease progression,
and developing more targeted treatments. Recent studies on
polymorphisms in thalassemia minor promise significant advancements
in the field of personalized medicine and targeted genetic therapies,
which can markedly enhance the quality of life for patients.
Methods: In this study, genetic data related to beta-thalassemia minor were retrieved from the NCBI database. The extracted sequences were analyzed and processed using bioinformatics approaches. Multiple sequence alignments and phylogenetic analyses were performed using MEGA software to investigate the genetic variations and polymorphisms associated with the disease.
Results: The analysis identified three key genes involved in beta-thalassemia minor: HBB, HBA1, and HBA2. For each of these genes, specific single nucleotide polymorphisms (SNPs) associated with the disease were identified and thoroughly examined. Using bioinformatics approaches, the data revealed the contribution of these genes to the disease, with HBB accounting for 21.42%, HBA1 for 14.28%, and HBA2 for 64.28% of the genetic variations observed.
Conclusion: This study provides a comprehensive bioinformatic overview of common polymorphisms associated with beta-thalassemia minor. The findings offer a deeper understanding of the genetic basis of the disease and may contribute to the development of SNP-based diagnostic tools or targeted gene therapies in the future.
Keywords: Beta-Thalassemia Minor - SNPs - Hemoglobinopathy