Skip to main content

Advertisement

Table 1 Consequences of frataxin depletion in various organismsa

From: Friedreich's ataxia: the vicious circle hypothesis revisited

Organism Increased ROS production/low glutathione Increased peroxidation products Hypersensitivity to oxidative insults Loss of ISP activity Mitochondrial iron overload References
Saccharomyces cerevisiae yeast +/++ +++ +++ +++ +++ [19]
Arabidopsis thaliana plant + n.d. +++ +++ ++ [69]
Caenorhabditis elegans worm n.d. n.d. +++ +++ n.d. [50]
Drosophila melanogaster fly (+) n.d. +++ +++ n.d. [70]
Mus musculus mouse (humanised) n.d. +++ n.d. +++ - [20]
Homo sapiens heart n.d. n.d. n.d. +++ ++ [4]
Homo sapiens lymphoblasts n.d. - +++ - - [14]
Homo sapiens skin fibroblasts + - +++ - - [9]
  1. aROS: reactive oxygen species; ISP: iron-sulphur cluster-containing protein; +++: hallmark of the disease; ++: present in most cases; +: present only in a subset of patients or late in the course of the disease; (+): reported occasionally; -: usually absent; n.d., not determined.