Cytoskeleton alterations of erythrocytes from patients with Fanconi's anemia

Walter Malorni, Elisabetta Straface, Giovanni Pagano, Daniela Monti, Adriana Zatterale, Domenico Del Principe, Irina B. Deeva, Claudio Franceschi, Roberta Masella, Ludmilla G. Korkina

Research output: Contribution to journalArticlepeer-review

Abstract

Fanconi's anemia (FA) is a very rare genetically heterogeneous disease which has been hypothesized to be defective in the detoxification of reactive oxygen species. In this work we report the results obtained by morphometric and biochemical analyses on the red blood cells (RBCs) from FA patients. With respect to RBCs from healthy donors the following changes have been detected: (i) a variety of ultrastructural alterations, mainly surface blebbing typical of acanthocytes and stomatocytes; (ii) a significant quantitative increase of these altered forms; (iii) modifications of spectrin cytoskeleton network; (iv) an altered redox balance, e.g. a decreased catalase activity and significant variations in the GSSG/GSH ratio. We hypothesize that remodeling of the redox state occurring in FA patients results in cytoskeleton- associated alterations of red blood cell integrity and function. (C) 2000 Federation of European Biochemical Societies.

Original languageEnglish
Pages (from-to)125-128
Number of pages4
JournalFEBS Letters
Volume468
Issue number2-3
DOIs
Publication statusPublished - Feb 25 2000

Keywords

  • Cytoskeleton
  • Erythrocyte
  • Fanconi's anemia
  • Reactive oxygen species

ASJC Scopus subject areas

  • Biochemistry
  • Biophysics
  • Molecular Biology

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