Τίτλος:
Decreased expression of membrane alpha 4 beta 1, alpha 5 beta 1
integrins and transferrin receptor on erythroblasts in splenectomized
patients with beta-thalassemia intermedia. Parallel assessment of serum
soluble transferrin receptors levels
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Dysfunction of cell membrane is a recognized consequence of the
pathogenetic process underlying the beta-thalassemia syndromes and it is
reasonable to hypothesize that surface structures crucial for the
development of erythroid lineage may also be affected. The study
included six adult splenectomized patients with beta-thalassemia
intermedia. Expression of alpha4beta1 integrin (CD49d/CD29), alpha5beta1
integrin (CD49e/CD29) and transferrin receptor (CD71) on peripheral
blood and bone marrow erythroblasts and on erythroid precursors grown in
vitro was studied by flow cytometry and immunocytochemistry. Serum
soluble transferrin receptor levels (sCD71) were also measured with
enzyme-linked immunosorbent assay. In beta-thalassemic patients,
significant reduction of CD49d, CD29 and CD71 expression was found in
peripheral blood nucleated red cells, compared to patients presenting
with erythroblasts in the circulation because of other diseases. Marrow
erythroblasts were also deficient for the same molecules against the
erythroblasts in iron deficiency anemia. All molecules tested were
greatly diminished on erythroid precursors developed in vitro from the
patients’ cells. Serum sCD71 levels were much higher in thalassemic
patients in comparison to both patients with iron deficiency anemia and
healthy individuals. The loss of certain integrins and CD71 from
erythroid precursors in beta-thalassemia intermedia could be attributed
to a generalized membrane dysfunction, perhaps affecting the integrity
of their transmembrane domains. The elevation of serum sCD71 levels may
be the result of the increased red cell lineage turnover or,
alternatively, may indicate increased shedding from the cells to prevent
iron overload. In any case, further molecular study of the membrane
components is warranted to provide a better understanding of the
pathogenetic process in beta-thalassemia syndromes.
Συγγραφείς:
Kossiva, L
Paterakis, G
Tassiopoulos, S
Papadhimitriou, SI
and Voukouti, E
Gligori, I
Rombos, Y
Περιοδικό:
Annals of Hematology
Λέξεις-κλειδιά:
integrins; transferrin receptors; beta-thalassemia intermedia
DOI:
10.1007/s00277-003-0708-z
