共查询到20条相似文献,搜索用时 0 毫秒
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B Giardina R Scatena M E Clementi L Cerroni S G Condò M Nuutinen O Brix 《Nature》1992,355(6363):777-778
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During human development there is a switch from fetal to adult haemoglobin formation, reflecting the differential expression of fetal (G gamma and A gamma) and adult (beta and delta) globin genes. Mutations that inhibit this switch produce variants of the syndrome of hereditary persistence of fetal haemoglobin (HPFH). Adult heterozygotes for these mutants produce 15-30% fetal haemoglobin (HbF) in their red cells. The general assumption is that the mutations result in a permanent switching on of gamma-globin genes. Here, however, we show that fetal globin expression can be turned off in cultures of HPFH cells by an uncharacterized factor in fetal sheep serum. This is the first demonstration that mutations affecting the developmental expression of globin genes can be modulated by exogenous factors. The findings raise the possibility that the phenotype of HPFH is not simply the direct result of mutations in or around globin genes but the consequence of the mutations on the interaction of globin genes with trans-acting regulatory factors. 相似文献
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Stereochemistry of cooperative effects in haemoglobin 总被引:136,自引:0,他引:136
M F Perutz 《Nature》1970,228(5273):726-739
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Functioning haemoglobin genes in non-nodulating plants 总被引:15,自引:0,他引:15
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Synthesis of haemoglobin Wayne in erythroid cells 总被引:2,自引:0,他引:2
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Haemoglobin is the prototype of an allosteric protein in which cooperative behaviour depends on interaction between unlike subunits. Here we present haematological and biochemical evidence that electrostatic interactions are an important determinant of haemoglobin assembly. Individuals heterozygous for positively charged beta-globin variants have a significantly lower proportion of abnormal haemoglobin than those with negatively charged variants. Moreover, these differences become more pronounced when alpha-thalassaemia is also present. Kinetic experiments using isolated chains indicate that the rate of assembly of the heterotetramer is influenced by alterations in surface charge. A simple electrostatic model is proposed in an attempt to explain these haematological and experimental findings. 相似文献