title: [Heterogeneity of globin protein synthesis in bone marrow cells of patients with homozygous beta-thalassemia from Tadzhikistan].
abstract: The synthesis of globin proteins in blood reticulocytes of homozygous beta-thalassemic patients from Tadzhikistan has been previously studied. beta-thalassemia with sharp repression of beta-globin protein synthesis (alpha/beta greater than 10) has been shown to be most representative for the region. In this work, the synthesis of globin proteins has been studied in bone marrow cells of homozygous beta-thalassemic patients. Comparison of data on globin synthesis in bone marrow cells and in blood reticulocytes of the patients has revealed that in some cases the disbalance of chain synthesis in both cell types is equal. In other cases the disbalance in bone marrow cells is less than in blood cells, indicating the instability of beta-globin mRNA that is partially degrading in the process of cell maturation. Homozygous beta-thalassemic cases with low content of Hb F in blood cells (5-10%), with substantial disbalance of alpha and beta-globin synthesis and marked production of gamma-globins in bone marrow cells and in blood reticulocytes are of special interest. It has been assumed that parallel to beta-thalassemia some instability of gamma-globin proteins takes place in these patients.
authors.forename: F Iu
authors.initials: FIu
authors.lastname: Nasyrova
authors.forename: A M
authors.initials: AM
authors.lastname: Mirakilova
authors.forename: I B
authors.initials: IB
authors.lastname: Ryzhova
authors.forename: R B
authors.initials: RB
authors.lastname: Sabzanova
authors.forename: S A
authors.initials: SA
authors.lastname: Limborskaia
mesh_terms: D000293:Adolescent; D001853:Bone Marrow / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D002850:Chromatography, Gel; D014644:Genetic Variation; D005914:Globins / Q000096:biosynthesis* / Q000235:genetics; D006720:Homozygote; D006801:Humans; D013620:Tajikistan; D013789:Thalassemia / Q000097:blood / Q000235:genetics* / Q000378:metabolism
chemical_list: D005914:Globins
vernacular_title: Geterogennost' sinteza globinovykh belkov v kletkakh kostnogo mozga bol'nykh gomozigotnoĭ beta-talassemieĭ iz Tadzhikistana.