作者: J. Rochette , J.E. Craig , S.L. Thein , J. Rochette
DOI: 10.1016/0268-960X(94)90109-0
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摘要: The synthesis of fetal hemoglobin (HbF) is normally reduced to very low levels less than 0.6% the total in adults. HbF restricted a sub-population erythrocytes termed 'F-cells'; 85% normal adult population have 0.3% 4.4% F-cells. and F-cells vary by more 10-fold adults; family studies show that these are genetically controlled but number nature genetic factors still poorly understood. may be increased adults inherited acquired disorders, accompanied an increase both amount per F-cell. clinical significance conditions with raised relates their interaction disorders such as sickle cell disease beta thalassaemia which can lead considerable amelioration severity. Study 'natural' mutants primarily associated has provided insight into understanding control globin gene regulation switching. Currently effort being channelled trials search for 'ideal' therapeutic agents could life minimal drug toxicity.