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Clinical Chemistry 44: 2172-2177, 1998;
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(Clinical Chemistry. 1998;44:2172-2177.)
© 1998 American Association for Clinical Chemistry, Inc.

Endocrinology and Metabolism

Hemoglobin Rambam (ß69[E13]Gly->Asp), a pitfall in the assessment of diabetic control: characterization by electrospray mass spectrometry and HPLC

E. Bissé1,a, N. Zorn2, A. Eigel3, M. Lizama1, P. Huaman-Guillen1, W. März1, A. Van Dorsselaer2, and H. Wieland1

1 Department of Clinical Chemistry, University Hospital, Hugstetterstrasse 55, D-79106 Freiburg, Germany.

2 Laboratoire de Spectrometrie de Masse Bio-Organique URA31, CNRS-Université Louis Pasteur, Faculté de Chimie, F-67008 Strasbourg, France.

3 Institut für Humangenetik Westfälische Wilhelms-Universität, D-48149 Münster, Germany.
a Author for correspondence. Fax 49-761-270-3444.

Hemoglobin (Hb) Rambam, or ß69[E13]Gly->Asp, has been identified in a German woman also suffering from non-insulin-dependent diabetes mellitus and chronic obstructive pulmonary disease. This is the first observation of this Hb variant in a German family thus far. The detailed evaluation of its structure using electrospray mass spectrometry revealed new minor glycohemoglobin components and showed that the attachment of glucose to the ß NH2 terminus occurred at an almost identical rate in both wild-type and mutant ß-chains. However, the introduction of a carboxyl group at ß69 seems to increase the glycation of {epsilon}-amino groups of lysine residues. The glycemic state in the propositus was well reflected by the total glycohemoglobin concentrations but not by the Hb A1C values, which did not reflect hemoglobin glycation in this patient. This case demonstrates that Hb A1C cannot be used reliably in the management of diabetic patients carrying Hb variants such as Hb Rambam. Functional studies of the whole blood of the heterozygous carrier demonstrated extremely low oxygen affinity, which may have been caused by increased 2,3-diphosphoglycerate related to chronic obstructive pulmonary disease and hyperthyroidism. None of the clinical symptoms could be directly associated to Hb Rambam.




The following articles in journals at HighWire Press have cited this article:


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 Clin. Chem.Home page
E. Bisse, C. Schauber, N. Zorn, T. Epting, A. Eigel, A. Van Dorsselaer, H. Wieland, J. Kister, and L. Kiger
Hemoglobin Gorwihl [{alpha}2{beta}25(A2)Pro->Ala], an Electrophoretically Silent Variant with Impaired Glycation
Clin. Chem., January 1, 2003; 49(1): 137 - 143.
[Abstract] [Full Text] [PDF]

Home page
 Clin. Chem.Home page
G. M. Clarke and T. N. Higgins
Laboratory Investigation of Hemoglobinopathies and Thalassemias: Review and Update
Clin. Chem., August 1, 2000; 46(8): 1284 - 1290.
[Abstract] [Full Text] [PDF]

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 J. Biol. Chem.Home page
E. Bisse, N. Zorn, I. Heinrichs, A. Eigel, A. Van Dorsselaer, H. Wieland, J. Kister, and M. C. Marden
Characterization of a New Electrophoretically Silent Hemoglobin Variant. Hb Saale OR alpha 2beta 2 84(EF8)Thr right-arrow Ala
J. Biol. Chem., July 7, 2000; 275(28): 21380 - 21384.
[Abstract] [Full Text] [PDF]


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