Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

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Chemical Compound Review:

FS4 iron; sulfanide

Synonyms:

Lobmayr, L. et al., Richards, T.A. et al., Kuhnke, G. et al., Gerber, J. et al., Simon, D. et al.

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High impact information on FS4

A function of mitochondria in cytosolic Fe/S protein maturation has been noted, but small amounts of some ISC components have been detected outside mitochondria [1].
Giardia and Trichomonas are eukaryotes without standard mitochondria but contain mitochondrial-type alpha-proteobacterium-derived iron-sulfur cluster (ISC) assembly proteins, located to mitosomes in Giardia and hydrogenosomes in Trichomonas [2].
Since the function of the mitochondrial iron-sulfur cluster (ISC) assembly machinery is also required for these two processes, Atm1p is thought to translocate a still unknown product of this pathway to the cytosol [3].
Here we demonstrate an incomplete shift of IRP1 to its ISC form in Friedreich ataxia (FRDA) fibroblasts, associated with decreased activities of ISC respiratory complexes [4].
It is caused by severely reduced levels of frataxin, a mitochondrial protein involved in iron-sulfur cluster (ISC) biosynthesis [5].

Gene context of FS4

In high-iron conditions, IRP1 incorporates an iron-sulfur cluster (ISC), which interferes with IRE binding and prevents intracellular iron accumulation [4].

References

The yeast scaffold proteins Isu1p and Isu2p are required inside mitochondria for maturation of cytosolic Fe/S proteins. Gerber, J., Neumann, K., Prohl, C., Mühlenhoff, U., Lill, R. Mol. Cell. Biol. (2004) [Pubmed]
Evolution of the Isd11-IscS complex reveals a single alpha-proteobacterial endosymbiosis for all eukaryotes. Richards, T.A., van der Giezen, M. Mol. Biol. Evol. (2006) [Pubmed]
Stimulation of the ATPase activity of the yeast mitochondrial ABC transporter Atm1p by thiol compounds. Kuhnke, G., Neumann, K., Mühlenhoff, U., Lill, R. Mol. Membr. Biol. (2006) [Pubmed]
Increased IRP1 activity in Friedreich ataxia. Lobmayr, L., Brooks, D.G., Wilson, R.B. Gene (2005) [Pubmed]
Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia. Simon, D., Seznec, H., Gansmuller, A., Carelle, N., Weber, P., Metzger, D., Rustin, P., Koenig, M., Puccio, H. J. Neurosci. (2004) [Pubmed]

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