The Sickle-Cell Fiber Revisited

Marilyn F Bishop; Frank A Ferrone

doi:10.3390/biom13030413

The Sickle-Cell Fiber Revisited

Biomolecules. 2023 Feb 22;13(3):413. doi: 10.3390/biom13030413.

Authors

Marilyn F Bishop¹, Frank A Ferrone²

Affiliations

¹ Department of Physics, Virginia Commonwealth University, Richmond, VA 23284, USA.
² Department of Physics, Drexel University, Philadelphia, PA 19104, USA.

Abstract

Sickle cell disease is the consequence of a single point mutation on the surface of the β chains of the hemoglobin molecule leading to the formation of rigid polymers that disrupt circulation. It has long been established that the polymers are comprised of seven pairs of double strands that are twisted replicas of the double strands found in crystals. Here, we review several newer developments that elaborate on that simple model and provide deeper insights into the process.

Keywords: double strand; electron microscopy; sickle cell polymer.

Publication types

Review
Research Support, Non-U.S. Gov't

MeSH terms

Anemia, Sickle Cell* / genetics
Hemoglobin, Sickle* / genetics
Hemoglobins / genetics
Humans
Point Mutation
Polymers

Substances

Hemoglobin, Sickle
Hemoglobins
Polymers

Grants and funding

This research was supported in part by a CURE grant from the Commonwealth of Pennsylvania and by a Seed Grant from the College of Humanities and Sciences, Virginia Commonwealth University.