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Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease


, : Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease. Disease Models & Mechanisms 5(2): 155-164

Iron-sulfur (Fe-S) clusters are ubiquitous cofactors composed of iron and inorganic sulfur. They are required for the function of proteins involved in a wide range of activities, including electron transport in respiratory chain complexes, regulatory sensing, photosynthesis and DNA repair. The proteins involved in the biogenesis of Fe-S clusters are evolutionarily conserved from bacteria to humans, and many insights into the process of Fe-S cluster biogenesis have come from studies of model organisms, including bacteria, fungi and plants. It is now clear that several rare and seemingly dissimilar human diseases are attributable to defects in the basic process of Fe-S cluster biogenesis. Although these diseases -which include Friedreich's ataxia (FRDA), ISCU myopathy, a rare form of sideroblastic anemia, an encephalomyopathy caused by dysfunction of respiratory chain complex I and multiple mitochondrial dysfunctions syndrome - affect different tissues, a feature common to many of them is that mitochondrial iron overload develops as a secondary consequence of a defect in Fe-S cluster biogenesis. This Commentary outlines the basic steps of Fe-S cluster biogenesis as they have been defined in model organisms. In addition, it draws attention to refinements of the process that might be specific to the subcellular compartmentalization of Fe-S cluster biogenesis proteins in some eukaryotes, including mammals. Finally, it outlines several important unresolved questions in the field that, once addressed, should offer important clues into how mitochondrial iron homeostasis is regulated, and how dysfunction in Fe-S cluster biogenesis can contribute to disease.

US$19.90

PMID: 22382365

DOI: 10.1242/dmm.009019


Other references

Goltsov, Alexey N., 1999: Electromagnetic-field-induced oscillations of the lipid domain structures in the mixed membranes. The effect of external electromagnetic field (EMF) on a percolation structure formed during phase separation in the mixed phospholipid membranes was studied by computer simulation. Decay of the percolation structure under electromagnetic radiation...

Geyer, S.; Jaunzeme, J.; Hillemanns, P., 2015: Cervical cancer screening in Germany: group-specific participation rates in the state of Niedersachsen (Lower Saxony). A study with health insurance data. The participation rates in cervical screenings are varying over different countries. This is only partly due to the availability of organized screening programs, as the rates are also varying between countries were such programs were implemented....

Caldwell J.; Sever P.S., 1974: The biochemical pharmacology of abused drugs part 1 amphetamines cocaine and lsd. Clinical Pharmacology & Therapeutics 16(4): 625-638

Gonzalez Peon, D.V.J., 1952: Problem of psychopaths in prisons; report of a case. Medicina 20(4 2): 271-276

Hevey, R.; Ling, C-Chun., 2017: Studies on the 6-homologation of β-D-idopyranosides. β-D-Idopyranosides are interesting sugars because of their unusual conformational flexibility in the pyranosyl ring, and also their β-1,2-cis-anomeric configuration. Here we report our studies of the regioselective opening of 4,6-O-benzylidene-p...

Irving, M.; Gwynne, K.; Angell, B.; Tennant, M.; Blinkhorn, A., 2016: Client perspectives on an Aboriginal community led oral health service in rural Australia. An oral health service was implemented, using a unique community development approach, for Northern NSW Australian Aboriginal communities in 2013-14. This study examined the views of children (and parents) who accessed the service, including: the...

Limpus, C.J.; Miller, J.D., 1993: Family Cheloniidae. Unknown

Haydar, Z.; Fleg, J.L.; Tobin, J.D.; Vaitkevicius, P.V.; Harman, S.M.; Blackman, M.R., 1996: Aging, not circulating IGF-1, influences aerobic capacity in healthy women and men. Journal of the American Geriatrics Society 44(9): S84

Serran, R.Beil, G.; Jinich, H.; Gutierre, S.Casa, J.I., 1961: The use of peritoneoscopy in clinical medicine. Nicaragua Medica 17: 70-94

Efem, S.E.; Aja, A., 1986: Surgeon's workshop: economic suction drainage in the third world. The Unical drain. Journal of the Royal College of Surgeons of Edinburgh 31(6): 370-371