Biosynthetic Studies and γ-Chain Composition in the Greek Type of Hereditary Persistence of Fetal Hemoglobin and in Its Association with β-Thalässemia

Autor:	M.A. Ciocca-Vasino, G. Saglio, Clara Camaschella, G. Balegno, Angelo Guerrasio, E. Barberis, D. Delponte
Rok vydání:	1979
Předmět:	Genetics congenital hereditary and neonatal diseases and abnormalities Hereditary persistence of fetal hemoglobin Thalassemia Beta thalassemia Heterozygote advantage Globin chain Hematology General Medicine Biology medicine.disease hemic and lymphatic diseases Fetal hemoglobin medicine
Zdroj:	Acta Haematologica. 61:272-277
ISSN:	1421-9662 0001-5792
DOI:	10.1159/000207671
Popis:	Hematological data, biosynthetic studies and gamma-chain structure of three heterozygotes for HPFH Greek type and of two heterozygotes for both HPFH and beta-thalassemia are reported. In the HPFH heterozygotes, hematological data were normal and globin chain synthesis balanced, while subjects carrying both HPFH and beta-thalassemia presented a thalassemic picture and the same degree of alpha/non-alpha-chain imbalance as the beta-thalassemia carrier belonging to the same family. The gamma-chain composition studies showed only the presence of Agamma-chains in HPFH; in the association HPFH/beta-thalassemia also some Ggamma and Tgamma were found. The mechanisms determining the high production of Hb F in the association HPFH/beta-thalassemia are discussed.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_________::4839f7c27491b211b0d3741d2cc678f7 https://doi.org/10.1159/000207671 Zobrazit plný text záznamu