Abstract
Hb Mont Saint-Aignan [β128(H6)Ala → Pro] is a mildly unstable variant, associated with hemolytic anemia, marked microcytosis and increased α/β biosynthetic ratio (1.55 versus1.1±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 β+-thalassemia-like expression of this variant are discussed.
Published Version
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