An essential function of the mitochondrial sulfhydryl oxidase Erv1p/ALR in the maturation of cytosolic Fe/S proteins

H Lange; T Lisowsky; J Gerber; U Mühlenhoff; G Kispal; R Lill

doi:10.1093/embo-reports/kve161

An essential function of the mitochondrial sulfhydryl oxidase Erv1p/ALR in the maturation of cytosolic Fe/S proteins

EMBO Rep. 2001 Aug;2(8):715-20. doi: 10.1093/embo-reports/kve161.

Authors

H Lange¹, T Lisowsky, J Gerber, U Mühlenhoff, G Kispal, R Lill

Affiliation

¹ Institut für Zytobiologie und Zytopathologie der Philipps-Universität Marburg, Robert-Koch-Strasse 5, 35033 Marburg, Germany.

Abstract

Biogenesis of Fe/S clusters involves a number of essential mitochondrial proteins. Here, we identify the essential Erv1p of Saccharomyces cerevisia mitochondria as a novel component that is specifically required for the maturation of Fe/S proteins in the cytosol, but not in mitochondria. Furthermore, Erv1p was found to be important for cellular iron homeostasis. The homologous mammalian protein ALR ('augmenter of liver regeneration'), also termed hepatopoietin, can functionally replace defects in Erv1p and thus represents the mammalian orthologue of yeast Erv1p. Previously, a fragment of ALR was reported to exhibit an activity as an extracellular hepatotrophic growth factor. Both Erv1p and full-length ALR are located in the mitochondrial intermembrane space and represent the first components of this compartment with a role in the biogenesis of cytosolic Fe/S proteins. It is likely that Erv1p/ALR operates downstream of the mitochondrial ABC transporter Atm1p/ABC7/Sta1, which also executes a specific task in this essential biochemical process.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Cytoplasm / chemistry
Cytoplasm / metabolism
Frataxin
Fungal Proteins / metabolism*
Genes, Reporter / genetics
Growth Substances / metabolism*
Humans
Iron-Binding Proteins*
Iron-Sulfur Proteins / metabolism*
Liver / chemistry
Liver / cytology
Mitochondria / enzymology*
Mitochondria / metabolism
Mitochondrial Proteins*
Oxidoreductases Acting on Sulfur Group Donors
Phosphotransferases (Alcohol Group Acceptor) / metabolism
Precipitin Tests
Proteins*
Recombinant Fusion Proteins / genetics
Recombinant Fusion Proteins / metabolism
Saccharomyces cerevisiae / cytology
Saccharomyces cerevisiae / enzymology*
Saccharomyces cerevisiae / metabolism
Saccharomyces cerevisiae Proteins*

Substances

Fungal Proteins
Growth Substances
Iron-Binding Proteins
Iron-Sulfur Proteins
Mitochondrial Proteins
Proteins
Recombinant Fusion Proteins
Saccharomyces cerevisiae Proteins
GFER protein, rat
Oxidoreductases Acting on Sulfur Group Donors
ERV1 protein, S cerevisiae
Phosphotransferases (Alcohol Group Acceptor)