[HTML][HTML] Iron regulatory protein-1 protects against mitoferrin-1-deficient porphyria
J Chung, SA Anderson, B Gwynn, KM Deck… - Journal of Biological …, 2014 - ASBMB
Mitochondrial iron is essential for the biosynthesis of heme and iron-sulfur ([Fe-S]) clusters in
mammalian cells. In developing erythrocytes, iron is imported into the mitochondria by
MFRN1 (mitoferrin-1, SLC25A37). Although loss of MFRN1 in zebrafish and mice leads to
profound anemia, mutant animals showed no overt signs of porphyria, suggesting that
mitochondrial iron deficiency does not result in an accumulation of protoporphyrins. Here,
we developed a gene trap model to provide in vitro and in vivo evidence that iron regulatory …
mammalian cells. In developing erythrocytes, iron is imported into the mitochondria by
MFRN1 (mitoferrin-1, SLC25A37). Although loss of MFRN1 in zebrafish and mice leads to
profound anemia, mutant animals showed no overt signs of porphyria, suggesting that
mitochondrial iron deficiency does not result in an accumulation of protoporphyrins. Here,
we developed a gene trap model to provide in vitro and in vivo evidence that iron regulatory …
