Iron-sulphur cluster biogenesis and mitochondrial iron homeostasis.
Iron-sulphur clusters are important cofactors for proteins that are involved in many cellular processes, including electron transport, enzymatic catalysis and regulation. The enzymes that catalyse the formation of iron-sulphur clusters are widely conserved from bacteria to humans. Recent studies in model systems and humans reveal that iron-sulphur proteins have important roles in mitochondrial iron homeostasis and in the pathogenesis of the human disease Friedreich ataxia.
Rouault
Tracey A
TA
National Institute of Child Health and Human Development, Cell Biology and Metabolism Branch, Bethesda, Maryland 20892, USA. trou@helix.nih.gov
Tong
Wing-Hang
WH
D000756:Anemia, Sideroblastic; D000818:Animals; D019143:Evolution, Molecular; D005621:Friedreich Ataxia / Q000473:pathology / Q000628:therapy; D006706:Homeostasis; D006801:Humans; D007501:Iron / Q000378:metabolism*; D019190:Iron Overload / Q000378:metabolism; D007506:Iron-Sulfur Proteins / Q000235:genetics / Q000378:metabolism*; D051379:Mice; D008928:Mitochondria / Q000378:metabolism*; D017434:Protein Structure, Tertiary; D015003:Yeasts / Q000378:metabolism
D007506:Iron-Sulfur Proteins; D007501:Iron