Abstract
Synthesis of α-globin and α-globin subunits of hemoglobin occurs at high levels during erythrocyte differentiation in a tightly controlled and co-ordinated fashion. Expression of α-globin is a fascinatingly complex process which has been meticulously defined in several recent studies, from chromatin modifications to Pol II recruitment. Following this, α-globin transcripts are processed and stabilized by a protein complex which binds the 3′ untranslated region. Transcription and stabilization contribute to high level expression of α-globin. However, translation of α-globin at levels exceeding α-globin expression damages cellular membranes and results in β-thalassemia. It is, therefore, crucial that α-globin proteins are properly folded and stabilized, processes which are dependent on the presence of haem and AHSP. The exceedingly well-characterized process of α-globin expression elegantly illustrates the complex interaction of factors which are required to balance necessary high expression against the negative impacts of overexpression.
Original language | English |
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Pages (from-to) | 1868-1876 |
Number of pages | 9 |
Journal | Haematologica |
Volume | 93 |
Issue number | 12 |
DOIs | |
Publication status | Published - 1 Dec 2008 |
Externally published | Yes |
Keywords
- α-globin
- Hemoglobin
- Thalassemia