TY - JOUR
T1 - Cytoskeleton alterations of erythrocytes from patients with Fanconi's anemia
AU - Malorni, Walter
AU - Straface, Elisabetta
AU - Pagano, Giovanni
AU - Monti, Daniela
AU - Zatterale, Adriana
AU - Del Principe, Domenico
AU - Deeva, Irina B.
AU - Franceschi, Claudio
AU - Masella, Roberta
AU - Korkina, Ludmilla G.
PY - 2000/2/25
Y1 - 2000/2/25
N2 - 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.
AB - 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.
KW - Cytoskeleton
KW - Erythrocyte
KW - Fanconi's anemia
KW - Reactive oxygen species
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U2 - 10.1016/S0014-5793(00)01187-X
DO - 10.1016/S0014-5793(00)01187-X
M3 - Article
C2 - 10692571
AN - SCOPUS:0033955246
VL - 468
SP - 125
EP - 128
JO - FEBS Letters
JF - FEBS Letters
SN - 0014-5793
IS - 2-3
ER -