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Characterization of Two Types of Fetal Hemoglobin: cy2'y2 and cy2*y2

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ETAL HEMOGLOBIN (HbF) contains two types of y F chain that differ in the amino acid residue at the 136th position: one type has alanine (Ay) as its residue and the other, glycine (‘y).’ There are usually two y-chain genes per chromosome, and the ‘y and Ay chains are the products of nonallelic tandem gene^.^.^ HbF isolated from red blood cells of newborns contains about 70% ‘y chain and 30% Ay chain, a proportion that is constant throughout fetal de~elopment.~.’ In contrast, the small amount of HbF found in adult red blood cells contains about 40% ‘7 chain and 60% chain.5 The transition from the fetal ‘yAy ratio to the “adult” ratio takes place during the first 10 months of Clinical severity in sickle cell disease is quite varied. The concept that elevation of HbF should favorably influence morbidity in persons with sickle cell anemia has been supported by both biochemical and clinical studies. Modification of clinical expression may also relate in part to the chromosomal background of

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ETAL HEMOGLOBIN (HbF) contains two types of y F chain that differ in the amino acid residue at the 136th position: one type has alanine (Ay) as its residue and the other, glycine (‘y).’ There are usually two y-chain genes per chromosome, and the ‘y and Ay chains are the products of nonallelic tandem gene^.^.^ HbF isolated from red blood cells of newborns contains about 70% ‘y chain and 30% Ay chain, a proportion that is constant throughout fetal de~elopment.~.’ In contrast, the small amount of HbF found in adult red blood cells contains about 40% ‘7 chain and 60% chain.5 The transition from the fetal ‘yAy ratio to the “adult” ratio takes place during the first 10 months of Clinical severity in sickle cell disease is quite varied. The concept that elevation of HbF should favorably influence morbidity in persons with sickle cell anemia has been supported by both biochemical and clinical studies. Modification of clinical expression may also relate in part to the chromosomal background of

Keywords

Fetal hemoglobinHaplotypeSickle cell anemiaInternal medicineBiologyFetusHemoglobinRed Cell

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