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Cytoskeleton alterations of erythrocytes from patients with Fanconi's anemia
Author(s) -
Malorni Walter,
Straface Elisabetta,
Pagano Giovanni,
Monti Daniela,
Zatterale Adriana,
Del Principe Domenico,
Deeva Irina B.,
Franceschi Claudio,
Masella Roberta,
Korkina Ludmilla G.
Publication year - 2000
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/s0014-5793(00)01187-x
Subject(s) - spectrin , cytoskeleton , fanconi anemia , red blood cell , biology , reactive oxygen species , catalase , glutathione , microbiology and biotechnology , oxidative stress , biochemistry , cell , gene , enzyme , dna repair
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.