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Frataxin Directly Stimulates Mitochondrial Cysteine Desulfurase by Exposing Substrate-binding Sites, and a Mutant Fe-S Cluster Scaffold Protein with Frataxin-bypassing Ability Acts Similarly*♦
Background: The cysteine desulfurase Nfs1 provides sulfur for Fe-S cluster biogenesis in mitochondria. Results: Frataxin or a mutant Fe-S scaffold protein (Isu1Sup) with frataxin-bypassing abilityExpand
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Endogenous Muscle Atrophy F-Box Mediates Pressure Overload–Induced Cardiac Hypertrophy Through Regulation of Nuclear Factor-&kgr;B
Rationale: Overexpression of muscle atrophy F-box (MAFbx/atrogin-1), an E3 ubiquitin ligase, induces proteasomal degradation in cardiomyocytes. The role of endogenous MAFbx in regulating cardiacExpand
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Rim2, a pyrimidine nucleotide exchanger, is needed for iron utilization in mitochondria.
Mitochondria transport and utilize iron for the synthesis of haem and Fe-S clusters. Although many proteins are known to be involved in these processes, additional proteins are likely to participate.Expand
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Histone H2A.z Is Essential for Cardiac Myocyte Hypertrophy but Opposed by Silent Information Regulator 2α*
In this study we have shown that the histone variant H2A.z is up-regulated during cardiac hypertrophy. Upon its knock-down with RNA interference, hypertrophy and the underlying increase inExpand
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Characterization of a Novel Cardiac Isoform of the Cell Cycle-related Kinase That Is Regulated during Heart Failure*
Myocardial infarction (MI) is often followed by heart failure (HF), but the mechanisms precipitating the transition to HF remain largely unknown. A genomic profile was performed in a monkey model ofExpand
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Further characterization of factor VIII-deficient mice created by gene targeting: RNA and protein studies.
Previously we created two strains of factor VIII-deficient mice by insertion of a neo gene into (1) the 3' end of exon 16 and (2) exon 17 of the factor VIII gene. Affected mice of both strains haveExpand
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Fe-S Cluster Biogenesis in Isolated Mammalian Mitochondria
Background: Fe-S cluster assembly in mitochondria involves generation of an activated form of sulfur called persulfide. Results: A novel experimental system tracks new Fe-S cluster synthesis inExpand
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Mitochondrial NADH Kinase, Pos5p, Is Required for Efficient Iron-Sulfur Cluster Biogenesis in Saccharomyces cerevisiae*
In Saccharomyces cerevisiae, the mitochondrial inner membrane readily allows transport of cytosolic NAD+, but not NADPH, to the matrix. Pos5p is the only known NADH kinase in the mitochondrialExpand
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In vitro characterization of a novel Isu homologue from Drosophila melanogaster for de novo FeS-cluster formation.
FeS-clusters are utilized by numerous proteins within several biological pathways that are essential for life. In eukaryotes, the primary FeS-cluster production pathway is the mitochondrialExpand
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Mutation in the Fe-S scaffold protein Isu bypasses frataxin deletion.
Frataxin is a conserved mitochondrial protein deficient in patients with Friedreich's ataxia. Frataxin has been implicated in control of iron homoeostasis and Fe-S cluster assembly. In yeast or humanExpand
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