Journal article
An α-chain modification rivals the effect of fetal hemoglobin in retarding the rate of sickle cell fiber formation
SCIENTIFIC REPORTS, v 13(1), 21997
11 Dec 2023
PMID: 38081985
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Adults with sickle cell disease bear a mutation in the beta-globin gene, leading to the expression of sickle hemoglobin (HbS; alpha 2 beta S2). Adults also possess the gene for gamma-globin, which is a component of fetal hemoglobin (HbF, alpha 2 gamma 2); however, gamma-chain expression normally ceases after birth. As HbF does not form the fibers that cause the disease, pharmacological and gene-modifying interventions have attempted to either reactivate expression of the gamma chain or introduce a gene encoding a modified beta chain having gamma-like character. Here, we show that a single-site modification on the alpha chain, alpha Pro114Arg, retards fiber formation as effectively as HbF. Because this addition to the repertoire of anti-sickling approaches acts independently of other modifications, it could be coupled with other therapies to significantly enhance their effectiveness.
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Details
- Title
- An α-chain modification rivals the effect of fetal hemoglobin in retarding the rate of sickle cell fiber formation
- Creators
- Frank A. Ferrone - Drexel University, Physics
- Publication Details
- SCIENTIFIC REPORTS, v 13(1), 21997
- Publisher
- NATURE PORTFOLIO; BERLIN
- Grant note
- No Statement Available
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Physics
- Web of Science ID
- WOS:001127268500046
- Scopus ID
- 2-s2.0-85179320722
- Other Identifier
- 991021861187604721
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- Collaboration types
- Domestic collaboration
- Web of Science research areas
- Biochemistry & Molecular Biology