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The deletions in the zeta-alpha globin gene cluster in two infants with the hemoglobin Bart's hydrops fetalis syndrome (homozygous alpha thalassemia 1) have been mapped by restriction endonuclease analysis using a zeta-specific probe. DNA from a Thai infant lacked the psi alpha 1 gene and both alpha genes, but the zeta genes were present. A Greek infant's DNA had also lost the 3' zeta 1 gene. Because zeta globin was synthesized in the infant's cord blood, this indicates that the 5' zeta 2 gene recently identified by Lauer et al. [Lauer, J., Shen, C. J. & Maniatis, T. (1980) Cell, in press] must be functional.


Journal article


Proc Natl Acad Sci U S A

Publication Date





3586 - 3589


*Chromosome Deletion Chromosome Mapping Crossing Over (Genetics) DNA Restriction Enzymes Edema/blood Fetal Hemoglobin/*genetics Genes, Structural Genes, Synthetic Globins/*genetics Human Infant, Newborn Infant, Newborn, Diseases/blood Nucleic Acid Hybridization Recombination, Genetic Thalassemia/blood/classification/*genetics