Go to JCI Insight
  • About
  • Editors
  • Consulting Editors
  • For authors
  • Publication ethics
  • Publication alerts by email
  • Advertising
  • Job board
  • Contact
  • Clinical Research and Public Health
  • Current issue
  • Past issues
  • By specialty
    • COVID-19
    • Cardiology
    • Gastroenterology
    • Immunology
    • Metabolism
    • Nephrology
    • Neuroscience
    • Oncology
    • Pulmonology
    • Vascular biology
    • All ...
  • Videos
    • Conversations with Giants in Medicine
    • Video Abstracts
  • Reviews
    • View all reviews ...
    • Complement Biology and Therapeutics (May 2025)
    • Evolving insights into MASLD and MASH pathogenesis and treatment (Apr 2025)
    • Microbiome in Health and Disease (Feb 2025)
    • Substance Use Disorders (Oct 2024)
    • Clonal Hematopoiesis (Oct 2024)
    • Sex Differences in Medicine (Sep 2024)
    • Vascular Malformations (Apr 2024)
    • View all review series ...
  • Viewpoint
  • Collections
    • In-Press Preview
    • Clinical Research and Public Health
    • Research Letters
    • Letters to the Editor
    • Editorials
    • Commentaries
    • Editor's notes
    • Reviews
    • Viewpoints
    • 100th anniversary
    • Top read articles

  • Current issue
  • Past issues
  • Specialties
  • Reviews
  • Review series
  • Conversations with Giants in Medicine
  • Video Abstracts
  • In-Press Preview
  • Clinical Research and Public Health
  • Research Letters
  • Letters to the Editor
  • Editorials
  • Commentaries
  • Editor's notes
  • Reviews
  • Viewpoints
  • 100th anniversary
  • Top read articles
  • About
  • Editors
  • Consulting Editors
  • For authors
  • Publication ethics
  • Publication alerts by email
  • Advertising
  • Job board
  • Contact
Top
  • View PDF
  • Download citation information
  • Send a comment
  • Terms of use
  • Standard abbreviations
  • Need help? Email the journal
  • Top
  • Abstract
  • Version history
  • Article usage
  • Citations to this article

Advertisement

Research Article Free access | 10.1172/JCI105670

Pathogenesis of Hemolytic Anemia in Homozygous Hemoglobin C Disease

Samuel Charache, C. Lockard Conley, David F. Waugh, Richard J. Ugoretz, and J. Richard Spurrell

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: 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: 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: 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: 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: PubMed | Google Scholar

Published November 1, 1967 - More info

Published in Volume 46, Issue 11 on November 1, 1967
J Clin Invest. 1967;46(11):1795–1811. https://doi.org/10.1172/JCI105670.
© 1967 The American Society for Clinical Investigation
Published November 1, 1967 - Version history
View PDF
Abstract

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.

Images.

Browse pages

Click on an image below to see the page. View PDF of the complete article

icon of scanned page 1795
page 1795
icon of scanned page 1796
page 1796
icon of scanned page 1797
page 1797
icon of scanned page 1798
page 1798
icon of scanned page 1799
page 1799
icon of scanned page 1800
page 1800
icon of scanned page 1801
page 1801
icon of scanned page 1802
page 1802
icon of scanned page 1803
page 1803
icon of scanned page 1804
page 1804
icon of scanned page 1805
page 1805
icon of scanned page 1806
page 1806
icon of scanned page 1807
page 1807
icon of scanned page 1808
page 1808
icon of scanned page 1809
page 1809
icon of scanned page 1810
page 1810
icon of scanned page 1811
page 1811
Version history
  • Version 1 (November 1, 1967): No description

Article tools

  • View PDF
  • Download citation information
  • Send a comment
  • Terms of use
  • Standard abbreviations
  • Need help? Email the journal

Metrics

  • Article usage
  • Citations to this article

Go to

  • Top
  • Abstract
  • Version history
Advertisement
Advertisement

Copyright © 2025 American Society for Clinical Investigation
ISSN: 0021-9738 (print), 1558-8238 (online)

Sign up for email alerts