HemoglobinBibba or α2136Pro β2, an unstable α chain abnormal hemoglobin. / Kleihauer, Enno F.; Reynolds, Cecelia A.; Dozy, Andrée M.; Wilson, Jerry B.; Moores, Russell R.; Berenson, M. P.; Wright, Claude Starr; Huisman, Titus H.J.In: BBA - Protein Structure, Vol. 154, No. 1, 22.01.1968, p. 220-222.
Research output: Contribution to journal › Article › peer-review
TY - JOUR
T1 - HemoglobinBibba or α2136Pro β2, an unstable α chain abnormal hemoglobin
AU - Kleihauer, Enno F.
AU - Reynolds, Cecelia A.
AU - Dozy, Andrée M.
AU - Wilson, Jerry B.
AU - Moores, Russell R.
AU - Berenson, M. P.
AU - Wright, Claude Starr
AU - Huisman, Titus H.J.
N1 - Funding Information: lyze the peptide bond between tile residues in position 136 and 137 (threonine) as is observed in the aT-I3 peptide of normal Hb-A. The replacement of leucine in position 136 of the a chain by proline has a profound influence on the stability of the abnormal hemoglobin. The substitution occurs in the C-terminal part of the H helix of the a chain and will likely disrupt the helix and change its direction, while as a result the interaction of the a and \[3 chains within the hemoglobin molecule may also be altered. Such a change in tertiary structure is also implicated by the differences in electrophoretic and chromatographic mobilities of the Hb-A and I-IbBibba, which are likely due to a change in the over all charge of the hemoglobin molecule and not to a substitution of an amino acid residue by another carrying a different charge. In this respect HbBibba differs from HbGenova (a2f122sLeu-~Pr°) which has an electrophoretic mobility similar to that of Hb-A, and resembles HbK~sln (a2f1298Val ~Met). This study was supported by U.S. Public Health Service grants HE-o5168 and FR-ooo6I-O5. E.F.K. was supported by U.S. Public Health Service grant i FOS-TW-II27-oI as an International Research Fellow and was on leave from the Department of Pediatrics, University of Munich, Germany.
PY - 1968/1/22
Y1 - 1968/1/22
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U2 - 10.1016/0005-2795(68)90274-2
DO - 10.1016/0005-2795(68)90274-2
M3 - Article
C2 - 5639009
AN - SCOPUS:0014426230
VL - 154
SP - 220
EP - 222
JO - Biochimica et Biophysica Acta - Proteins and Proteomics
JF - Biochimica et Biophysica Acta - Proteins and Proteomics
SN - 1570-9639
IS - 1