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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.