Pathophysiology of beta thalassemia--a guide to molecular therapies.

doi:10.1182/asheducation-2005.1.31

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Article

Pathophysiology of beta thalassemia--a guide to molecular therapies.

Hematology. American Society of Hematology. Education Program - 1 Jan 2005

Thein Swee Lay

Abstract excerpt

The central mechanism underlying the pathophysiology of the beta thalassemias can be related to the deleterious effects of imbalanced globin chain synthesis on erythroid maturation and survival. An imbalance of the alpha/non-alpha globin chains leads to an excess of unmatched alpha globin which precipitates out, damaging membrane structures leading to accelerated apoptosis and premature destruction of the...

Topics

Erythropoiesis
Fetal Hemoglobin
Genetic Therapy
Genetic Variation
Genotype
Globins
Hemolysis
Heterozygote
Humans
Phenotype
Quantitative Trait Loci
alpha-Globins
alpha-Thalassemia
beta-Thalassemia

Source

Open in PubMed

Identifiers

PMID: 16304356
DOI: 10.1182/asheducation-2005.1.31

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