|
|
 Previous Article | Table of Contents | Next Article 
Blood, 1953, Vol. 8, No. 11, pp. 1008-1023.
© 1953 American Society of Hematology, Inc.
Studies on Abnormal Hemoglobins
VIII. The Gelling Phenomenon of Sickle Cell Hemoglobin: Its
Biologic and Diagnostic Significance
KARL SINGER M.D.1 and
LILY SINGER M.S.1
1 Department of Hematologic Research, Medical Research Institute, Michael
Reese Hospital, Chicago, Ill.
1. When sufficiently concentrated sickle cell hemoglobin containing solutions
are exposed to a constant stream of CO2 gas, the hemolysates gel. This gelling
phenomenon is indicative of the presence of S hemoglobin and cannot be obtained
with any other type of human hemoglobin in the absence of S pigment. The
lowest S hemoglobin concentration (Gm. per cent) of a hemolysate at which the
gelling phenomenon can still be elicited is designated as its lowest gelling point.
2. A simple apparatus was developed to analyze the gelling phenomenon under
standardized conditions. It could be shown that the lowest gelling points of
hemolysates prepared from erythrocytes of the sickle cell trait (containing A +
S hemoglobins), of the "C variant" (containing C + S hemoglobins), and from
sickle cell anemia cells (containing S + F hemoglobins) differ distinctly. Further
experiments suggest that the presence of A hemoglobin decreases the minimal
amount of S pigment required for gel formation, and that type C hemoglobin
reduces this amount even further. F hemoglobin seems to exert no significant
influence on the gelling phenomenon. Serum albumin is also capable of decreasing
the amount of S hemoglobin required for gelation.
3. A sickled erythrocyte is visualized as an S hemoglobin tactoid or gel, specifically influenced by the companion pigment which interacts with the S compound.
Thus, in the sickle cell trait, a positive sickling test is not only caused by the
presence of S hemoglobin, but also by its interaction with A hemoglobin. Only
in the sickle cell anemia cells does sickling seem to depend solely upon the interaction of the S hemoglobin molecules.
4. The readily demonstrable differences of the lowest gelling points of hemolysates prepared from the various types of sickling red cells form the basis of the
diagnostic gelling test which distinguishes sharply between sickle cell anemia and
sickle cell trait erythrocytes. By this procedure atypical cases of sickle cell disease,
for example, those whose erythrocytes contain C hemoglobin, may also be
detected.
Submitted on April 21, 1953
Accepted on May 25, 1953
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
R. Grubina, S. Basu, M. Tiso, D. B. Kim-Shapiro, and M. T. Gladwin
Nitrite Reductase Activity of Hemoglobin S (Sickle) Provides Insight into Contributions of Heme Redox Potential Versus Ligand Affinity
J. Biol. Chem.,
February 8, 2008;
283(6):
3628 - 3638.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
V. L. Lew and R. M. Bookchin
Ion Transport Pathology in the Mechanism of Sickle Cell Dehydration
Physiol Rev,
January 1, 2005;
85(1):
179 - 200.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. E.C. McMahon and E. J. Mark
Case 34-1997- A 22-Year-Old Man with a Sickle Cell Crisis and Sudden Death
N. Engl. J. Med.,
October 30, 1997;
337(18):
1293 - 1301.
[Full Text]
[PDF]
|
|
|
|
|
|
|
W. Eaton and J Hofrichter
The biophysics of sickle cell hydroxyurea therapy
Science,
May 26, 1995;
268(5214):
1142 - 1143.
[PDF]
|
|
|
|
|
|
|
J. D. Corbett, W. E. Mickols, and M. F. Maestre
Effect of Hemoglobin Concentration on Nucleation and Polymer Formation in Sickle Red Blood Cells
J. Biol. Chem.,
February 10, 1995;
270(6):
2708 - 2715.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. Moffat
Gelation of Sickle Cell Hemoglobin: Effects of Hybrid Tetramer Formation in Hemoglobin Mixtures
Science,
July 19, 1974;
185(4147):
274 - 277.
[Abstract]
[PDF]
|
|
|
|
|
|
|
J. F. Bertles, R. Rabinowitz, and J. Dobler
Hemoglobin Interaction: Modification of Solid Phase Composition in the Sickling Phenomenon
Science,
July 24, 1970;
169(3943):
375 - 377.
[Abstract]
[PDF]
|
|
|
|
|
|
|
R. C. GRIGGS and J. W. HARRIS
The Biophysics of the Variants of Sickle-Cell Disease
Arch Intern Med,
March 1, 1956;
97(3):
315 - 326.
[Abstract]
[PDF]
|
|
|
|
|
|
|
J. W. HARRIS, H. H. BREWSTER, T. H. HAM, and W. B. CASTLE
Studies on the Destruction of Red Blood Cells: X. The Biophysics and Biology of Sickle-Cell Disease
Arch Intern Med,
February 1, 1956;
97(2):
145 - 168.
[Abstract]
[PDF]
|
|
|
|
|
|
|
C.-S. WRIGHT, D. S. MABRY, R. D. CARR, and A. M. PERRY
SURVEY OF THE 1953 HEMATOLOGY LITERATURE
Arch Intern Med,
November 1, 1954;
94(5):
806 - 845.
[Abstract]
[PDF]
|
|
|
|
|
