Iron regulatory protein-1 protects against mitoferrin-1-deficient porphyria.

doi:10.1074/jbc.M114.547778

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Article

Iron regulatory protein-1 protects against mitoferrin-1-deficient porphyria.

The Journal of biological chemistry - 14 Mar 2014

Chung Jacky, Anderson Sheila A, Gwynn Babette, Deck Kathryn M, Chen Michael J, Langer Nathaniel B, Shaw George C, Huston Nicholas C, Boyer Leah F, Datta Sumon, Paradkar Prasad N, Li Liangtao, Wei Zong, Lambert Amy J, Sahr Kenneth, Wittig Johannes G, Chen Wen, Lu Wange, Galy Bruno, Schlaeger Thorsten M, Hentze Matthias W, Ward Diane M, Kaplan Jerry, Eisenstein Richard S, Peters Luanne L, Paw Barry H

Abstract excerpt

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...

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PMID: 24509859
DOI: 10.1074/jbc.M114.547778
PMCID: PMC3953295

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