Title : Iron regulatory protein 1 sustains mitochondrial iron loading and function in frataxin deficiency.

Pub. Date : 2015 Feb 3

PMID : 25651183






8 Functional Relationships(s)
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1 Iron regulatory protein 1 sustains mitochondrial iron loading and function in frataxin deficiency. Iron aconitase 1 Mus musculus
2 Using a mouse model of hepatic FXN deficiency in combination with mice deficient for iron regulatory protein 1 (IRP1), a key regulator of cellular iron metabolism, we show that IRP1 activation in conditions of Fe-S deficiency increases the available cytosolic labile iron pool. Iron aconitase 1 Mus musculus
3 Using a mouse model of hepatic FXN deficiency in combination with mice deficient for iron regulatory protein 1 (IRP1), a key regulator of cellular iron metabolism, we show that IRP1 activation in conditions of Fe-S deficiency increases the available cytosolic labile iron pool. Iron aconitase 1 Mus musculus
4 Using a mouse model of hepatic FXN deficiency in combination with mice deficient for iron regulatory protein 1 (IRP1), a key regulator of cellular iron metabolism, we show that IRP1 activation in conditions of Fe-S deficiency increases the available cytosolic labile iron pool. Iron aconitase 1 Mus musculus
5 Using a mouse model of hepatic FXN deficiency in combination with mice deficient for iron regulatory protein 1 (IRP1), a key regulator of cellular iron metabolism, we show that IRP1 activation in conditions of Fe-S deficiency increases the available cytosolic labile iron pool. Iron aconitase 1 Mus musculus
6 Using a mouse model of hepatic FXN deficiency in combination with mice deficient for iron regulatory protein 1 (IRP1), a key regulator of cellular iron metabolism, we show that IRP1 activation in conditions of Fe-S deficiency increases the available cytosolic labile iron pool. Iron aconitase 1 Mus musculus
7 Surprisingly, our data indicate that IRP1 activation sustains mitochondrial iron supply and function rather than driving detrimental iron overload. Iron aconitase 1 Mus musculus
8 Our results uncover an unexpected protective role of IRP1 in pathological conditions associated with altered Fe-S metabolism. Iron aconitase 1 Mus musculus