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Glycogen branching enzyme controls cellular iron homeostasis via Iron Regulatory Protein 1 and mitoNEET
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Glycogen branching enzyme controls cellular iron homeostasis via Iron Regulatory Protein 1 and mitoNEET
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Journal Article
Glycogen branching enzyme controls cellular iron homeostasis via Iron Regulatory Protein 1 and mitoNEET
2019
Overview
Iron Regulatory Protein 1 (IRP1) is a bifunctional cytosolic iron sensor. When iron levels are normal, IRP1 harbours an iron-sulphur cluster (holo-IRP1), an enzyme with aconitase activity. When iron levels fall, IRP1 loses the cluster (apo-IRP1) and binds to iron-responsive elements (IREs) in messenger RNAs (mRNAs) encoding proteins involved in cellular iron uptake, distribution, and storage. Here we show that mutations in the
Drosophila
1,4-Alpha-Glucan Branching Enzyme (
AGBE
) gene cause porphyria.
AGBE
was hitherto only linked to glycogen metabolism and a fatal human disorder known as glycogen storage disease type IV. AGBE binds specifically to holo-IRP1 and to mitoNEET, a protein capable of repairing IRP1 iron-sulphur clusters. This interaction ensures nuclear translocation of holo-IRP1 and downregulation of iron-dependent processes, demonstrating that holo-IRP1 functions not just as an aconitase, but throttles target gene expression in anticipation of declining iron requirements.
Higher organisms regulate cellular iron concentrations through Iron Regulatory Proteins (IRPs), which regulate specific messenger RNAs. Here Huynh et al. show that IRP1 requires a Glycogen Branching Enzyme for proper function, and that IRP1 has additional regulatory roles in cell nuclei.
Publisher
Nature Publishing Group UK,Nature Publishing Group,Nature Portfolio
Subject
1,4-alpha-Glucan Branching Enzyme - genetics
/ 1,4-alpha-Glucan Branching Enzyme - metabolism
/ 13/1
/ 13/106
/ 13/44
/ 14/19
/ 14/63
/ 38/39
/ 38/91
/ 42/89
/ 64/24
/ 82/58
/ Active Transport, Cell Nucleus
/ Animals
/ Clusters
/ Drosophila Proteins - genetics
/ Drosophila Proteins - metabolism
/ Endocrine Glands - metabolism
/ Enzymes
/ Gene Expression Regulation - genetics
/ Glucan
/ Glycogen
/ Humanities and Social Sciences
/ Iron
/ Iron Regulatory Protein 1 - genetics
/ Iron Regulatory Protein 1 - metabolism
/ Iron-Sulfur Proteins - metabolism
/ Mitochondrial Proteins - genetics
/ Mitochondrial Proteins - metabolism
/ Mutation
/ Proteins
/ Science
/ Sulfur
