Cells from individuals with SOD-1 associated familial amyotrophic lateral sclerosis do not have an increased susceptibility to radiation-induced freeradical production or DNA damage
Np. Mithal et al., Cells from individuals with SOD-1 associated familial amyotrophic lateral sclerosis do not have an increased susceptibility to radiation-induced freeradical production or DNA damage, J NEUR SCI, 164(1), 1999, pp. 89-92
Oxidative stress may play a role in the pathogenesis of familial amyotrophi
c lateral sclerosis (FALS). Superoxide dismutases (SODs) are enzymes that c
an influence free radical processes in irradiated cells and there is some e
vidence that manipulation of SODs can affect survival of cells after radiat
ion treatments. SOD-1 associated FALS mutants may have an altered radiation
response due to an enhanced generation of hydroxyl radicals or a compromis
ed ability to neutralize free radicals. We have investigated the ability of
the lymphoblastoid cell lines from FALS patients with SOD-1 gene mutations
, patients with sporadic ALS and controls to handle oxidative stress induce
d by ionising radiation by measuring levels of intracellular reactive oxyge
n species and production of DNA double-strand breaks. Levels of reactive ox
ygen species, expressed as the slope of the relative fluorescence of a radi
cal-reactive fluorochrome, in the cells from familial ALS patients with SOD
-1 gene mutations (2.14+/-1.06 Gy(-1)) and patients with sporadic ALS (1.38
+/-0.21 Gy(-1)) were not significantly different from the controls (1.54+/-
0.39 Gy(-1)). No significant difference was observed in the production of D
NA double-strand breaks between three groups. The ability of lymphoblastoid
cells from FALS patients with SOD-1 gene mutations to scavenge radiation-i
nduced free radicals is not compromised nor is their ability to protect DNA
damage induced by ionising radiation. (C) 1999 Elsevier Science B.V. All r
ights reserved.