دورية أكاديمية
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Surprising Structural and Functional Properties of Favism Erythrocytes Are Linked to Special Metabolic Regulation: A Cell Aging Study.

التفاصيل البيبلوغرافية
العنوان: Surprising Structural and Functional Properties of Favism Erythrocytes Are Linked to Special Metabolic Regulation: A Cell Aging Study.
المؤلفون: Dinarelli S; Italian National Research Council (CNR), Institute for the Structure of the Matter (ISM), Via fosso del Cavaliere 100, 00133 Rome, Italy., Longo G; Italian National Research Council (CNR), Institute for the Structure of the Matter (ISM), Via fosso del Cavaliere 100, 00133 Rome, Italy., Germanova-Taneva S; Bulgarian Academy of Sciences (BAS), Institute of Biophysics and Biomedical Engineering, G. Bonchev Str. 21, 1113 Sofia, Bulgaria., Todinova S; Bulgarian Academy of Sciences (BAS), Institute of Biophysics and Biomedical Engineering, G. Bonchev Str. 21, 1113 Sofia, Bulgaria., Krumova S; Bulgarian Academy of Sciences (BAS), Institute of Biophysics and Biomedical Engineering, G. Bonchev Str. 21, 1113 Sofia, Bulgaria., Girasole M; Italian National Research Council (CNR), Institute for the Structure of the Matter (ISM), Via fosso del Cavaliere 100, 00133 Rome, Italy.
المصدر: International journal of molecular sciences [Int J Mol Sci] 2022 Dec 30; Vol. 24 (1). Date of Electronic Publication: 2022 Dec 30.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: MDPI Country of Publication: Switzerland NLM ID: 101092791 Publication Model: Electronic Cited Medium: Internet ISSN: 1422-0067 (Electronic) Linking ISSN: 14220067 NLM ISO Abbreviation: Int J Mol Sci Subsets: MEDLINE
أسماء مطبوعة: Original Publication: Basel, Switzerland : MDPI, [2000-
مواضيع طبية MeSH: Favism*/genetics , Anemia, Hemolytic* , Vicia faba* , Glucosephosphate Dehydrogenase Deficiency*/genetics, Humans ; Erythrocytes/pathology ; Cellular Senescence
مستخلص: Favism uniquely arises from a genetic defect of the Glucose-6 Phosphate Dehydrogenase (G6PD) enzyme and results in a severe reduction of erythrocytes' (RBCs) reducing power that impairs the cells' ability to respond to oxidative stresses. After exposure to fava beans or a few other drugs, the patients experience acute hemolytic anemia due to RBCs' lysis both intra and extra-vascularly. In the present paper, we compared selected biochemical, biophysical, and ultra-morphological properties of normal RBCs and cells from favism patients measured along cellular aging. Along the aging path, the cells' characteristics change, and their structural and functional properties degrade for both samples, but with different patterns and effectors that have been characterized in biophysical and biochemical terms. In particular, the analysis revealed distinct metabolic regulation in G6DP-deficient cells that determines important peculiarities in the cell properties during aging. Remarkably, the initial higher fragility and occurrence of structural/morphological alterations of favism cells develop, with longer aging times, into a stronger resistance to external stresses and higher general resilience. This surprisingly higher endurance against cell aging has been related to a special mechanism of metabolic regulation that permits lower energy consumption in environmental stress conditions. Our results provided a direct and coherent link between the RBCs' metabolic regulation and the cell properties that would not have been possible to establish without an investigation performed during aging. The consequences of this new knowledge, in particular, can be discussed in a more general context, such as understanding the role of the present findings in determining the characteristics of the favism pathology as a whole.
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معلومات مُعتمدة: CNR-BAS bilateral programme 2016-2018 National Research Council; КП-06-Н31/8, Competition for financial support of basic research projects - 2019 Bulgarian Academy of Sciences
فهرسة مساهمة: Keywords: AFM; cell aging; erythrocytes; favism; membrane roughness; metabolic adaptation; redox homeostasis; thermal stability of proteins
تواريخ الأحداث: Date Created: 20230108 Date Completed: 20230110 Latest Revision: 20230111
رمز التحديث: 20231215
مُعرف محوري في PubMed: PMC9820584
DOI: 10.3390/ijms24010637
PMID: 36614084
قاعدة البيانات: MEDLINE
الوصف
تدمد:1422-0067
DOI:10.3390/ijms24010637