TY - JOUR
T1 - β-Like globin RNA sequences in hemoglobin Lepore disease
AU - Giglioni, B.
AU - Comi, P.
AU - Taramelli, R.
AU - Pozzoli, M.
AU - Zanollo, A.
AU - Ottolenghi, S.
AU - Gianni, A. M.
PY - 1979
Y1 - 1979
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=0018743772&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0018743772&partnerID=8YFLogxK
U2 - 10.1111/j.1432-1033.1979.tb12993.x
DO - 10.1111/j.1432-1033.1979.tb12993.x
M3 - Article
C2 - 446479
AN - SCOPUS:0018743772
VL - 95
SP - 527
EP - 531
JO - European Journal of Biochemistry
JF - European Journal of Biochemistry
SN - 0014-2956
IS - 3
ER -