Hemoglobin Brest [beta 127 (H5)Gln----Lys] a new unstable human hemoglobin variant located at the alpha 1 beta 1 interface with specific electrophoretic behavior.
Hb Brest [beta 127 (H5)Gln----Lys] is a new unstable variant located at the alpha 1 beta 1 interface at the same position as Hb Complutense [beta 127(H5)Gln----Glu]. In each of these, the substitution produces a distinct alteration in charge, yet both variants move with Hb A in conventional electrophoresis. This peculiar electrophoretical behavior may be due to the molecular position of the modified residue, which is deeply buried inside the tetramer.
Baudin-Chich
V
V
INSERM U299, Le Kremlin-Bicêtre, France.
Wajcman
H
H
Gombaud-Saintonge
G
G
Arous
N
N
Riou
J
J
Brière
J
J
Galacteros
F
F
D000595:Amino Acid Sequence; D000745:Anemia, Hemolytic, Congenital / Q000097:blood / Q000235:genetics*; D001797:Blood Protein Electrophoresis; D002648:Child; D004588:Electrophoresis, Cellulose Acetate; D005914:Globins / Q000235:genetics*; D006453:Hemoglobinopathies / Q000097:blood / Q000235:genetics*; D006455:Hemoglobins, Abnormal / Q000235:genetics* / Q000302:isolation & purification; D006801:Humans; D008297:Male; D008958:Models, Molecular; D008969:Molecular Sequence Data; D011487:Protein Conformation
D006455:Hemoglobins, Abnormal; C056397:hemoglobin Brest; D005914:Globins