HB Melusine [α114(GH2)PRO→SER]: A New Neutral Hemoglobin Variant

Content Provider	Scilit
Author	Wajcman, H. Kalmes, G. Groff, P. Prome, D. Riou, J. Galacteros, F.
Copyright Year	1993
Description	Hb Melusine [α114(GH2)Pro→Ser] was found in an Algerian patient during a systematic screening for hemoglobinopathies performed in Luxembourg. The abnormal hemoglobin was suspected when a thickening of the Hb A band was observed by isoelectrofocusing. The mutant hemoglobin was silent in all other electrophoretic methods used for presumptive diagnosis with the exception of globin electrophoresis in the presence of Triton X-100. This technique revealed an α chain considerably more hydrophobic than normal. The structural abnormality of Hb Melusine concerns position α114(GH2) that belongs to a cluster of hydrophobic residues localized in the N-terminal half of the αT-12b tryptic peptide. It has been shown in the case of another variant of that position (Hb Nouakchott), that the replacement of the Pro GH2 by a Leu was responsible for a dramatic increase in the retention time of the a polypeptide chain during reversed phase high performance liquid chromatography, much higher than that reported for similar substitutions in other regions of the hemoglobin molecule.
Related Links	https://www.tandfonline.com/doi/pdf/10.3109/03630269308997494
Ending Page	405
Page Count	9
Starting Page	397
ISSN	03630269
e-ISSN	1532432X
DOI	10.3109/03630269308997494
Journal	Hemoglobin
Issue Number	5
Volume Number	17
Language	English
Publisher	Informa UK Limited
Publisher Date	1993-01-01
Access Restriction	Open
Subject Keyword	Journal: Hemoglobin Anesthesiology Hb Melusine 114 Hemoglobin Variant New Neutral Neutral Hemoglobin Gh2
Content Type	Text
Resource Type	Article
Subject	Biochemistry (medical) Hematology Clinical Biochemistry Genetics (clinical)