Human embryonic e globin chains were isolated from lysates of cultured human leukemic K562 cells induced with hemin. Mice were immunized with globin chains, and a murine hybridoma cell line was established that secreted monoclonal anti-c globin antibody. A sensitive and specific slot blot immunoassay for e globin chains has been developed. #{128} Globin chains were shown to be present in hemoly...

After screening a bacterial artificial chromosome of human genomic DNA library with human HS-40, zeta-, alpha-, and theta-globin probes, a 110-kb clone bearing the whole human alpha-globin gene cluster was obtained and rare restriction endonuclease mapping was performed. The bacterial artificial chromosome DNA was isolated, and transgenic mice were generated. Three founders were detected from 3...

Expression of fetal γ-globin in adulthood ameliorates symptoms of β-hemoglobinopathies by compensating for the mutant β-globin. Reactivation of the silenced γ-globin gene is therefore of substantial clinical interest. To study the regulation of γ-globin expression, we created the GG mice, which carry an intact 183-kb human β-globin locus modified to express enhanced green fluorescent protein (e...

Thalassemia is the world’s most common form of inherited anemia, and in economically undeveloped countries still accounts for tens of thousands of premature deaths every year. The accumulation of free excess α-globin chains in red blood cells and their precursors, as a result of the decreased production of b-globin, is believed to be the main pathophysiological mechanism leading to hemolytic an...

BACKGROUND Blood-based biomarker discovery with gene expression profiling has been hampered by interference from endogenous, highly abundant alpha- and beta-globin transcripts. We describe a means to quantify the interference of globin transcripts on profiling and the effectiveness of globin transcript mitigation by (a) defining and characterizing globin interference, (b) reproducing globin int...

Phylogenetic reconstructions of the beta-globin gene family in vertebrates have revealed that developmentally regulated systems of hemoglobin synthesis have been reinvented multiple times in independent lineages. For example, the functional differentiation of embryonic and adult beta-like globin genes occurred independently in birds and mammals. In both taxa, the embryonic beta-globin gene is e...

The a globin gene locus of the common gibbon (Hylobates lar) was isolated, and it contains two closely linked a globin genes that share the same arrangement as that found for the homologous genes of other catarrhine primates. The nucleotide sequences of the gibbon a globin genes were determined and compared with the a globin gene sequences from other primate species (human, chimpanzee, oranguta...

BACKGROUND beta-thalassemia occurs from the imbalanced globin chain synthesis due to the absence or inadequate beta-globin chain production. The excessive unbound alpha-globin chains precipitate in erythroid precursors and mature red blood cells leading to ineffective erythropoiesis and hemolysis. DESIGN AND METHODS In vitro globin chain synthesis in reticulocytes from different types of thal...

A high-titer amphotropic retroviral vector containing the neomycin resistance gene and a hybrid gamma-beta genomic human globin gene has been constructed. Mouse erythroleukemia cells infected with this virus were found to contain the full transcriptional unit of the transferred human globin gene by Southern blot analysis. These cells contain normally initiated, spliced, and terminated human glo...