β-Like globin RNA sequences in hemoglobin Lepore disease

B. Giglioni, P. Comi, R. Taramelli, M. Pozzoli, A. Zanollo, S. Ottolenghi, A. M. Gianni

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Abstract

A Southern Italian patient homozygous for hemoglobin Lepore disease synthesizes approximately 3% Lopore δβ-globin chains (relative to α chains) in the reticulocytes. Measurement of β-like RNA squences by hydridization to complementary DNA specific for β-globin demonstrates a low level (1-2% relative to α sequences) of these sequences in cytoplasmic RNA from reticulocytes or spleen cells, suggesting that the Lepore gene is expressed into mRNA at a lower extent than normal α or β genes; the comparison with the level of β-like sequences found in nuclear RNA (6-8%) further supports this conclusion and indicates, in addition, that Lepore RNA might be degraded at a faster rate than normal. 2-3% β-like sequences are found in nuclear RNA in three cases of homozygous β 0-thalassemia, setting the highest possible estimate for the δ-RNA level; this figure suggests that the 'δ-promotor'-dependent Lepore δβ gene is somehow more actively expressed than the δ gene.

Original languageEnglish
Pages (from-to)527-531
Number of pages5
JournalEuropean Journal of Biochemistry
Volume95
Issue number3
DOIs
Publication statusPublished - 1979

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ASJC Scopus subject areas

  • Biochemistry

Cite this

Giglioni, B., Comi, P., Taramelli, R., Pozzoli, M., Zanollo, A., Ottolenghi, S., & Gianni, A. M. (1979). β-Like globin RNA sequences in hemoglobin Lepore disease. European Journal of Biochemistry, 95(3), 527-531. https://doi.org/10.1111/j.1432-1033.1979.tb12993.x