Περίληψη:
Hb Mont Saint-Aignan [beta 128(H6)Ala –> Pro] is a mildly unstable
variant, associated with hemolytic anemia, marked microcytosis and
increased alp biosynthetic ratio (1.55 versus 1.1 +/-0.1 in the
control). The abnormal chain was isolated by selective precipitation
with isopropanol and the structural modification determined by protein
chemistry methods (reversed phase high performance liquid chromatography
and mass spectrometry). Possible mechanisms underlying the beta
(+)-thalassemia-like expression of this variant are discussed.
Συγγραφείς:
Wajcman, H
Lahary, A
Prome, D
Kister, J
Riou, J and
Godart, C
Prehu, C
Traeger-Synodinos, J
Papassotiriou, I and
Galacteros, F
