Hb Matera [beta 55(D6)Met----Lys]: a new unstable hemoglobin variant in an Italian family.
Hb Matera is a newly discovered, slow-moving, beta chain variant observed in four members of an Italian family. The variant had a decreased stability in heat and isopropanol tests and numerous red cells contained inclusion bodies. Structural analysis revealed that methionine at position beta 55 was replaced by lysine. Hb Matera's instability is due to an interference in the alpha 1-beta 1 contact of the hemoglobin molecule.
Sciarratta
G V
GV
Centro della Microcitemia, Ospedali Galliera, Genova, Italy.
Ivaldi
G
G
D000328:Adult; D000595:Amino Acid Sequence; D005914:Globins / Q000235:genetics*; D006455:Hemoglobins, Abnormal / Q000235:genetics* / Q000302:isolation & purification; D006801:Humans; D008297:Male; D010449:Peptide Mapping; D011487:Protein Conformation
D006455:Hemoglobins, Abnormal; C065342:hemoglobin Matera; D005914:Globins