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The Sickle-Cell Fiber Revisited
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 twis...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10046210/ https://www.ncbi.nlm.nih.gov/pubmed/36979347 http://dx.doi.org/10.3390/biom13030413 |
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author | Bishop, Marilyn F. Ferrone, Frank A. |
author_facet | Bishop, Marilyn F. Ferrone, Frank A. |
author_sort | Bishop, Marilyn F. |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-10046210 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-100462102023-03-29 The Sickle-Cell Fiber Revisited Bishop, Marilyn F. Ferrone, Frank A. Biomolecules Review 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. MDPI 2023-02-22 /pmc/articles/PMC10046210/ /pubmed/36979347 http://dx.doi.org/10.3390/biom13030413 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Bishop, Marilyn F. Ferrone, Frank A. The Sickle-Cell Fiber Revisited |
title | The Sickle-Cell Fiber Revisited |
title_full | The Sickle-Cell Fiber Revisited |
title_fullStr | The Sickle-Cell Fiber Revisited |
title_full_unstemmed | The Sickle-Cell Fiber Revisited |
title_short | The Sickle-Cell Fiber Revisited |
title_sort | sickle-cell fiber revisited |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10046210/ https://www.ncbi.nlm.nih.gov/pubmed/36979347 http://dx.doi.org/10.3390/biom13030413 |
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