期刊
HAEMATOLOGICA-THE HEMATOLOGY JOURNAL
卷 93, 期 12, 页码 1868-1876出版社
FERRATA STORTI FOUNDATION
DOI: 10.3324/haematol.13490
关键词
hemoglobin; alpha-globin; thalassemia
类别
资金
- National Health and Medical Research Council
- Murdoch Children's Research Institute
- Thalassaemia Society of Victoria
Synthesis of alpha-globin and alpha-globin subunits of hemoglobin occurs at high levels during erythrocyte differentiation in a tightly controlled and co-ordinated fashion. Expression of alpha-globin is a fascinatingly complex process which has been meticulously defined in several recent studies, from chromatin modifications to Pol II recruitment. Following this, alpha-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 alpha-globin. However, translation of alpha-globin at levels exceeding alpha-globin expression damages cellular membranes and results in beta-thalassemia. It is, therefore, crucial that alpha-globin proteins are properly folded and stabilized, processes which are dependent on the presence of haem and AHSP The exceedingly well-characterized process of alpha-globin expression elegantly illustrates the complex interaction of factors which are required to balance necessary high expression against the negative impacts of overexpression.
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