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Further Evidence for a Genetic Basis of Haemoglobin H Disease from Newborn Offspring of Patients
Naturevolume 223, pages59–60 (1969)Cite this article
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Abstract
HAEMOGLOBIN H disease is usually a mild to moderate, sometimes severe, thalassaemic disease, characterized by the presence of haemoglobin (Hb) H in addition to Hb A, and intraerythrocytic inclusion bodies1–3. Hb H is usually not detectable in either parent of the patients. The genetic basis of the disease was a subject for speculation until Hb H was shown to consist entirely of β-polypeptide chains4, with a molecular formula of β4, while Hb A is α2β2. It is now realized that Hb H (β4) is connected with α-thalassaemia5 which, by suppressing α-chain synthesis, leads to polymerization of the unconjugated β-chains to a tetrameric form. For the same reason Hbγ4 (Bart's) and Hb δ4 occur as a result of α-chain suppression6,7. Both haemoglobins can be detected in the people with Hb H disease.
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Department of Medicine, Faculty of Medicine and Siriraj Hospital, Mahidol University, Bangkok, Thailand
SUPA NA-NAKORN, PRAWASE WASI, MALIDA PORNPATKUL & SA-NGA POOTRAKUL
- SUPA NA-NAKORN
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- MALIDA PORNPATKUL
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NA-NAKORN, S., WASI, P., PORNPATKUL, M.et al. Further Evidence for a Genetic Basis of Haemoglobin H Disease from Newborn Offspring of Patients.Nature223, 59–60 (1969). https://doi.org/10.1038/223059a0
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