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Research Article Free access | 10.1172/JCI105670
Department of Medicine, The Johns Hopkins University and Hospital, Baltimore, Maryland
Department of Biology, Massachusetts Institute of Technology, Boston, Massachusetts
‡Henry Strong Denison Scholar for 1966-67.
Address requests for reprints to Dr. Samuel Charache, The Johns Hopkins Hospital, Baltimore, Md. 21205.
*Received for publication 19 May 1967 and in revised form 12 July 1967.
These studies were supported by a postdoctoral fellowship, 5-F2-HE-15, 899-02, from the National Heart Institute, Research Grant HE-02799, from the National Heart Institute, a Graduate Training Grant T1-AM-5260, from the National Institute of Arthritis and Metabolic Diseases, Grant GM-05410, from the Division of General Medical Sciences of the U. S. Public Health Service, and a Grant from the Molecular Biology Program of the National Science Foundation.
Purchase and modification of the viscometer was made possible by a special grant from the Medical Foundation, Inc., of Boston, Mass.
Find articles by Charache, S. in: JCI | PubMed | Google Scholar
Department of Medicine, The Johns Hopkins University and Hospital, Baltimore, Maryland
Department of Biology, Massachusetts Institute of Technology, Boston, Massachusetts
‡Henry Strong Denison Scholar for 1966-67.
Address requests for reprints to Dr. Samuel Charache, The Johns Hopkins Hospital, Baltimore, Md. 21205.
*Received for publication 19 May 1967 and in revised form 12 July 1967.
These studies were supported by a postdoctoral fellowship, 5-F2-HE-15, 899-02, from the National Heart Institute, Research Grant HE-02799, from the National Heart Institute, a Graduate Training Grant T1-AM-5260, from the National Institute of Arthritis and Metabolic Diseases, Grant GM-05410, from the Division of General Medical Sciences of the U. S. Public Health Service, and a Grant from the Molecular Biology Program of the National Science Foundation.
Purchase and modification of the viscometer was made possible by a special grant from the Medical Foundation, Inc., of Boston, Mass.
Find articles by Conley, C. in: JCI | PubMed | Google Scholar
Department of Medicine, The Johns Hopkins University and Hospital, Baltimore, Maryland
Department of Biology, Massachusetts Institute of Technology, Boston, Massachusetts
‡Henry Strong Denison Scholar for 1966-67.
Address requests for reprints to Dr. Samuel Charache, The Johns Hopkins Hospital, Baltimore, Md. 21205.
*Received for publication 19 May 1967 and in revised form 12 July 1967.
These studies were supported by a postdoctoral fellowship, 5-F2-HE-15, 899-02, from the National Heart Institute, Research Grant HE-02799, from the National Heart Institute, a Graduate Training Grant T1-AM-5260, from the National Institute of Arthritis and Metabolic Diseases, Grant GM-05410, from the Division of General Medical Sciences of the U. S. Public Health Service, and a Grant from the Molecular Biology Program of the National Science Foundation.
Purchase and modification of the viscometer was made possible by a special grant from the Medical Foundation, Inc., of Boston, Mass.
Find articles by Waugh, D. in: JCI | PubMed | Google Scholar
Department of Medicine, The Johns Hopkins University and Hospital, Baltimore, Maryland
Department of Biology, Massachusetts Institute of Technology, Boston, Massachusetts
‡Henry Strong Denison Scholar for 1966-67.
Address requests for reprints to Dr. Samuel Charache, The Johns Hopkins Hospital, Baltimore, Md. 21205.
*Received for publication 19 May 1967 and in revised form 12 July 1967.
These studies were supported by a postdoctoral fellowship, 5-F2-HE-15, 899-02, from the National Heart Institute, Research Grant HE-02799, from the National Heart Institute, a Graduate Training Grant T1-AM-5260, from the National Institute of Arthritis and Metabolic Diseases, Grant GM-05410, from the Division of General Medical Sciences of the U. S. Public Health Service, and a Grant from the Molecular Biology Program of the National Science Foundation.
Purchase and modification of the viscometer was made possible by a special grant from the Medical Foundation, Inc., of Boston, Mass.
Find articles by Ugoretz, R. in: JCI | PubMed | Google Scholar
Department of Medicine, The Johns Hopkins University and Hospital, Baltimore, Maryland
Department of Biology, Massachusetts Institute of Technology, Boston, Massachusetts
‡Henry Strong Denison Scholar for 1966-67.
Address requests for reprints to Dr. Samuel Charache, The Johns Hopkins Hospital, Baltimore, Md. 21205.
*Received for publication 19 May 1967 and in revised form 12 July 1967.
These studies were supported by a postdoctoral fellowship, 5-F2-HE-15, 899-02, from the National Heart Institute, Research Grant HE-02799, from the National Heart Institute, a Graduate Training Grant T1-AM-5260, from the National Institute of Arthritis and Metabolic Diseases, Grant GM-05410, from the Division of General Medical Sciences of the U. S. Public Health Service, and a Grant from the Molecular Biology Program of the National Science Foundation.
Purchase and modification of the viscometer was made possible by a special grant from the Medical Foundation, Inc., of Boston, Mass.
Find articles by Spurrell, J. in: JCI | PubMed | Google Scholar
Published November 1, 1967 - More info
Hemoglobin C is less soluble than hemoglobin A in red cells, in hemolysates, and in dilute phosphate buffer. Its relative insolubility may be explained by electrostatic interactions between positively charged β6-lysyl groups and negatively charged groups on adjacent molecules. Red cells from patients with homozygous hemoglobin C (CC) disease exhibit aberrant physical properties which suggest that the cells are more rigid than normal erythrocytes. They pass through membrane filters less readily than normal red cells do, and their viscosity is higher than that of normal cells. Differences from normal cells are exaggerated if mean corpuscular hemoglobin concentration (MCHC) is increased, by suspension in hypertonic salt solution. Increased rigidity of CC cells, by accelerating their fragmentation, may be responsible for formation of microspherocytes. These small dense cells are exceptionally rigid, and probably are even more susceptible to fragmentation and sequestration. Rigidity of CC cells can be attributed to a “precrystalline” state of intracellular hemoglobin, in which crystallization does not occur, although the MCHC exceeds the solubility of hemoglobin in hemolysates.
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