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- W2140529822 abstract "Hb Redondo [β92(F8) His→Asn] illustrates how post-translational structural modifications may modify the phenotypic expression of an unstable hemoglobin. This variant was found in a Portuguese patient suffering from a chronic hemolytic anemia. The electrophoretic pattern demonstrated that it occurred in two forms, both being semi-hemoglobins: the fastest one migrating like HbS and the other like HbA2; after a few days of storage at 4°C the intensity of the slowest Hb fraction decreased while that of the other increased proportionally. In both cases, the RP-HPLC analysis of the tryptic digest of the aminoethylated β chains demonstrated the presence of an abnormal βT10 peptide carrying a His→Asx substitution. Microsequence study of these two peptides demonstrated that the slowest abnormal Hb fraction had a β92 His → Asn substitution and the fastest a His → Asp at the same site. All these results suggest that the β92 His → Asn variant loses readily its heme group and that a deamidation occurs rapidly in vitro, yielding a β92 Asp semi-hemoglobin. The oxygen affinity of the patient's red blood cells was increased, leading to a stimulation of erythropoiesis and to a macrocytic hemolytic disease." @default.
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- W2140529822 date "1991-11-01" @default.
- W2140529822 modified "2023-10-18" @default.
- W2140529822 title "Hemoglobin redondo [β 92(F8) His → Asn]: An unstable hemoglobin variant associated with heme loss which occurs in two forms" @default.
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- W2140529822 doi "https://doi.org/10.1002/ajh.2830380308" @default.
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