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Iron-sulfur cluster scaffold homolog

HML, IscU, HML-2, NFU
Iron-sulfur (Fe-S) clusters are necessary for several mitochondrial enzymes and other subcellular compartment proteins. They contain sulfur and iron, and are created via several steps that include cysteine desulfurases, iron donors, chaperones, and scaffold proteins. This gene encodes the two isomeric forms, ISCU1 and ISCU2, of the Fe-S cluster scaffold protein. Mutations in this gene have been found in patients with myopathy with severe exercise intolerance and myoglobinuria. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: CAN, hormone-receptor, ACID, HAD, V1a
Papers using HML antibodies
The PyMOL molecular graphics system
Supplier
Markley John L et al., In BMC Biochemistry, 1995
... IscU as a scaffold for iron-sulfur cluster biosynthesis: sequential assembly of [2Fe-2S] ...
Papers on HML
Disease-Causing SDHAF1 Mutations Impair Transfer of Fe-S Clusters to SDHB.
New
Impact
Rouault et al., Bethesda, United States. In Cell Metab, Jan 2016
SDHAF1 transiently binds to aromatic peptides of SDHB through an arginine-rich region in its C terminus and specifically engages a Fe-S donor complex, consisting of the scaffold, holo-ISCU, and the co-chaperone-chaperone pair, HSC20-HSPA9, through an LYR motif near its N-terminal domain.
Determination of sequences required for HERV-K transduction and its recognition by foreign retroviral virions.
New
McClure et al., London, United Kingdom. In J Virol, Jan 2016
UNASSIGNED: Sequences necessary for transduction of HERV-Kcon, a consensus of the HERV-K(HML-2) family were analysed and found to reside in the leader/gag region.
Mitochondrial Hspa9/Mortalin regulates erythroid differentiation via iron-sulfur cluster assembly.
New
Cortopassi et al., Davis, United States. In Mitochondrion, Jan 2016
HSPA9 interacts with and stabilizes the mitochondrial ISC biogenesis proteins frataxin, Nfs1, ISCU, and Nfu.
New insights in the acute toxic/genotoxic effects of CuO nanoparticles in the in vivo Drosophila model.
New
Marcos et al., Barcelona, Spain. In Nanotoxicology, Jan 2016
In this point it should be emphasized the novelty of using genes such as Duox, Upd3, PPO2 and Hml to determine injury on the intestinal barrier.
The Conformation of Yeast Chromosome III Is Mating Type Dependent and Controlled by the Recombination Enhancer.
New
Dekker et al., Worcester, United States. In Cell Rep, Jan 2016
Mating-type switching in yeast occurs through gene conversion between the MAT locus and one of two silent loci (HML or HMR) on opposite ends of the chromosome.
Identification and spontaneous immune targeting of an endogenous retrovirus K envelope protein in the Indian rhesus macaque model of human disease.
New
Sacha et al., Beaverton, United States. In Retrovirology, Dec 2015
FINDINGS: Here, we identified the rhesus macaque equivalent of the biologically active human ERV-K (HML-2), simian ERV-K (SERV-K1), which retains intact open reading frames for both Gag and Env on chromosome 12 in the macaque genome.
Intrinsic retroviral reactivation in human preimplantation embryos and pluripotent cells.
New
Impact
Wysocka et al., Stanford, United States. In Nature, Jul 2015
The most recently acquired human ERV is HERVK(HML-2), which repeatedly infected the primate lineage both before and after the divergence of the human and chimpanzee common ancestor.
Assembly of Fe/S proteins in bacterial systems: Biochemistry of the bacterial ISC system.
Review
New
Ollagnier de Choudens et al., Grenoble, France. In Biochim Biophys Acta, Jun 2015
Some of the most exciting recent highlights relating to this system include structural and functional characterization of binary and ternary complexes involved in Fe/S cluster formation on the scaffold protein IscU.
Tangled web of interactions among proteins involved in iron-sulfur cluster assembly as unraveled by NMR, SAXS, chemical crosslinking, and functional studies.
Review
New
Markley et al., Madison, United States. In Biochim Biophys Acta, Jun 2015
Our investigations over the past five years have identified two functional conformational states for the scaffold protein (IscU) and have shown that the other ISC proteins that interact with IscU prefer to bind one conformational state or the other.
Mating-type Gene Switching in Saccharomyces cerevisiae.
Review
New
Haber et al., In Microbiol Spectr, Apr 2015
Donor preference is controlled by a cis-acting recombination enhancer located near HML.
HypoxamiRs and cancer: from biology to targeted therapy.
Review
Ivan et al., Sydney, Australia. In Antioxid Redox Signal, 2014
Several miR-210 target genes, including iron-sulfur (Fe-S) cluster scaffold protein (ISCU) and glycerol-3-phosphate dehydrogenase 1-like (GPD1L), have been correlated with prognosis in an inverse fashion to miR-210, suggesting that their down- regulation by miR-210 occurs in vivo and contributes to tumor growth.
Mammalian target of rapamycin coordinates iron metabolism with iron-sulfur cluster assembly enzyme and tristetraprolin.
Review
Wang et al., China. In Nutrition, 2014
mTOR can form two multiprotein complexes that consist of mTOR complex 1 (mTORC1) and mTOR complex 2. Recent advances clearly demonstrate that mTORC1 can phosphorylate iron-sulfur cluster assembly enzyme ISCU and affect iron-sulfur clusters assembly.
Cochaperone binding to LYR motifs confers specificity of iron sulfur cluster delivery.
Impact
Rouault et al., Bethesda, United States. In Cell Metab, 2014
Iron sulfur (Fe-S) clusters, preassembled on the ISCU scaffold, are transferred to target proteins or to intermediate scaffolds by a dedicated chaperone-cochaperone system.
Heterotrifunctional chemical cross-linking mass spectrometry confirms physical interaction between human frataxin and ISU.
GeneRIF
Busenlehner et al., Tuscaloosa, United States. In Biochemistry, 2012
Photoreactive heterotrifunctional chemical cross-linking confirmed the interaction between frataxin and ISCU in the presence of iron and validated that transient interactions can be covalently trapped with this method.
Targeting of macrophage galactose-type C-type lectin (MGL) induces DC signaling and activation.
GeneRIF
Nuti et al., Roma, Italy. In Eur J Immunol, 2012
MGL engagement improved DC performance as antigen-presenting cells, promoting the upregulation of maturation markers, a decrease in phagocytosis, an enhancement of motility, and most importantly an increase in antigen-specific CD8(+) T-cell activation.
The defective splicing caused by the ISCU intron mutation in patients with myopathy with lactic acidosis is repressed by PTBP1 but can be derepressed by IGF2BP1.
GeneRIF
Holmberg et al., Umeå, Sweden. In Hum Mutat, 2012
The defective splicing caused by the ISCU intron mutation in patients with myopathy with lactic acidosis is repressed by PTBP1 but can be derepressed by IGF2BP1.
A fatal mitochondrial disease is associated with defective NFU1 function in the maturation of a subset of mitochondrial Fe-S proteins.
GeneRIF
Lill et al., Barcelona, Spain. In Am J Hum Genet, 2011
A fatal mitochondrial disease is associated with defective NFU1 function in the maturation of a subset of mitochondrial Fe-S proteins
Mutations in iron-sulfur cluster scaffold genes NFU1 and BOLA3 cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes.
GeneRIF
Robinson et al., Toronto, Canada. In Am J Hum Genet, 2011
Mutations in iron-sulfur cluster scaffold genes NFU1 and BOLA3 cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes [case report]
Functions of mitochondrial ISCU and cytosolic ISCU in mammalian iron-sulfur cluster biogenesis and iron homeostasis.
Impact
GeneRIF
Rouault et al., Bethesda, United States. In Cell Metab, 2006
Functional analysis of the mitochondrial and cytosolic isoforms of the human Fe-S cluster scaffold protein, ISCU.
Iron-sulfur cluster biosynthesis: toward an understanding of cellular machinery and molecular mechanism.
Review
Impact
Cowan et al., Columbus, United States. In Acc Chem Res, 2004
The IscU (or ISU) family of proteins serves a key role as scaffolding proteins on which [2Fe-2S] building blocks are assembled prior to transfer to final apo target proteins.
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