http://www.cnr.it/ontology/cnr/individuo/prodotto/ID26371
Hb G-San Jose variant levels correlate with alpha-thalassemia genotypes. (Articolo in rivista)
- Type
- Label
- Hb G-San Jose variant levels correlate with alpha-thalassemia genotypes. (Articolo in rivista) (literal)
- Anno
- 2002-01-01T00:00:00+01:00 (literal)
- Alternative label
Lacerra G., Fiorito M., Pagano L., Testa R., Magnano C., Medulla E., Carestia C.. (2002)
Hb G-San Jose variant levels correlate with alpha-thalassemia genotypes.
in Hemoglobin
(literal)
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- Lacerra G., Fiorito M., Pagano L., Testa R., Magnano C., Medulla E., Carestia C.. (literal)
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- Rivista
- Note
- ISI Web of Science (WOS) (literal)
- Titolo
- Hb G-San Jose variant levels correlate with alpha-thalassemia genotypes. (literal)
- Abstract
- Hb G-San Jose or beta7(A4)Glu-->Gly has been reported in Southern
Italian or Mexican families. We have studied four families from Sicily
and Campania, Southern Italy. In six carriers, the hemoglobin variant
level ranged from 32 to 38%. In four double heterozygotes for Hb
G-San Jose and alpha-thalassemia the variant level showed a strong
correlation with the alpha-thalassemia genotype. In fact, the variant
level was 15% when interacting with the - (alpha)20.5/alphaalpha,
19.6% with the alphaalpha/alphaPoly Aalpha, and 24.8% with
alphaalpha/alpha(-5) ntalpha genotypes. In two double heterozygotes
for Hb G-San Jose and beta+ -IVS-I-6 (T-->C) the hemoglobin variant
level was 67%. These data show that the reduced synthesis of alpha
chains causes drastic reduction of probability to form Hb G-San Jose
in favor of the formation of Hb A. Moreover, this reduction, (i)
correlates with the type of alpha-thalassemia genotype and with the
degree of the alpha chain deficiency, and (ii) is, most probably, more
marked than the degree of alpha chain reduction. The minor affinity of
the beta chain variant for the alpha chains associated with the
reduced synthesis of the alpha chains is probably the principal cause
of the variant hemoglobin reduction. Moreover, the rapid removal of
the abnormal chains by proteolytic enzymes must have an essential
role in order to reduce the chain variant pool. These conclusions are
in agreement with the results obtained in reticulocyte and in vitro
recombination experiments. (literal)
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