Jerry Kaplan

Summary

Affiliation: University of Utah
Country: USA

Publications

  1. pmc The essential nature of iron usage and regulation
    Jerry Kaplan
    Department of Pathology, University of Utah School of Medicine, Salt Lake City, UT 84132, USA
    Curr Biol 23:R642-6. 2013
  2. ncbi request reprint Spinocerebellar ataxias due to mitochondrial defects
    Jerry Kaplan
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Neurochem Int 40:553-7. 2002
  3. ncbi request reprint Iron-dependent metabolic remodeling in S. cerevisiae
    Jerry Kaplan
    Department of Pathology, School of Medicine University of Utah, Salt Lake City, UT 84132 2501, USA
    Biochim Biophys Acta 1763:646-51. 2006
  4. doi request reprint The molecular basis of iron overload disorders and iron-linked anemias
    Jerry Kaplan
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84312, USA
    Int J Hematol 93:14-20. 2011
  5. ncbi request reprint Mechanisms of cellular iron acquisition: another iron in the fire
    Jerry Kaplan
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Cell 111:603-6. 2002
  6. pmc Human ESCRT-II complex and its role in human immunodeficiency virus type 1 release
    Charles Langelier
    Department of Biochemistry, 15 N Medical Drive East, Room 4100, University of Utah School of Medicine, Salt Lake City, UT 84112 5650, USA
    J Virol 80:9465-80. 2006
  7. pmc A single amino acid change in the yeast vacuolar metal transporters ZRC1 and COT1 alters their substrate specificity
    Huilan Lin
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    J Biol Chem 283:33865-73. 2008
  8. pmc The molecular mechanism of hepcidin-mediated ferroportin down-regulation
    Ivana De Domenico
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Mol Biol Cell 18:2569-78. 2007
  9. pmc Identification of FRA1 and FRA2 as genes involved in regulating the yeast iron regulon in response to decreased mitochondrial iron-sulfur cluster synthesis
    Attila Kumanovics
    Department of Pathology and Departments of Biochemistry and Medicine, School of Medicine, University of Utah, Salt Lake City Utah 84132, USA
    J Biol Chem 283:10276-86. 2008
  10. ncbi request reprint The role of LIP5 and CHMP5 in multivesicular body formation and HIV-1 budding in mammalian cells
    Diane McVey Ward
    University of Utah Health Sciences Center, Department of Pathology, Salt Lake City, Utah 84132, USA
    J Biol Chem 280:10548-55. 2005

Research Grants

Collaborators

Detail Information

Publications83

  1. pmc The essential nature of iron usage and regulation
    Jerry Kaplan
    Department of Pathology, University of Utah School of Medicine, Salt Lake City, UT 84132, USA
    Curr Biol 23:R642-6. 2013
    ....
  2. ncbi request reprint Spinocerebellar ataxias due to mitochondrial defects
    Jerry Kaplan
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Neurochem Int 40:553-7. 2002
    ..It is hypothesized that the biochemical feature common to these ataxias is increased oxidant damage either through increased oxidants or decreased anti-oxidants...
  3. ncbi request reprint Iron-dependent metabolic remodeling in S. cerevisiae
    Jerry Kaplan
    Department of Pathology, School of Medicine University of Utah, Salt Lake City, UT 84132 2501, USA
    Biochim Biophys Acta 1763:646-51. 2006
    ..In this review, we describe how the budding yeast Saccharomyces cerevisiae utilizes multiple mechanisms to optimize iron usage under iron limiting conditions...
  4. doi request reprint The molecular basis of iron overload disorders and iron-linked anemias
    Jerry Kaplan
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84312, USA
    Int J Hematol 93:14-20. 2011
    ..Genetic disorders of iron overload of iron-linked anemia can be explained by changes in the level of hepcidin or ferroportin and of the ability of ferroportin to be internalized by hepcidin...
  5. ncbi request reprint Mechanisms of cellular iron acquisition: another iron in the fire
    Jerry Kaplan
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Cell 111:603-6. 2002
    ..Two reports in the current issue of Molecular Cell suggest an unexpected candidate for the Tf-independent system...
  6. pmc Human ESCRT-II complex and its role in human immunodeficiency virus type 1 release
    Charles Langelier
    Department of Biochemistry, 15 N Medical Drive East, Room 4100, University of Utah School of Medicine, Salt Lake City, UT 84112 5650, USA
    J Virol 80:9465-80. 2006
    ..These observations indicate that there are probably multiple pathways for protein sorting/MVB vesicle formation in human cells and that HIV-1 does not utilize an ESCRT-II-dependent pathway to leave the cell...
  7. pmc A single amino acid change in the yeast vacuolar metal transporters ZRC1 and COT1 alters their substrate specificity
    Huilan Lin
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    J Biol Chem 283:33865-73. 2008
    ..These mutations are within the second hydrophobic domain of the transporters and show the essential nature of this domain in the specificity of metal transport...
  8. pmc The molecular mechanism of hepcidin-mediated ferroportin down-regulation
    Ivana De Domenico
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Mol Biol Cell 18:2569-78. 2007
    ..Depletion of proteins involved in multivesicular body trafficking (Endosome Sorting Complex Required for Transport proteins), by small-interfering RNA, reduces the trafficking of Fpn-green fluorescent to the lysosome...
  9. pmc Identification of FRA1 and FRA2 as genes involved in regulating the yeast iron regulon in response to decreased mitochondrial iron-sulfur cluster synthesis
    Attila Kumanovics
    Department of Pathology and Departments of Biochemistry and Medicine, School of Medicine, University of Utah, Salt Lake City Utah 84132, USA
    J Biol Chem 283:10276-86. 2008
    ..These results show that the Fra-Grx complex is an intermediate between the production of mitochondrial Fe-S clusters and transcription of the iron regulon...
  10. ncbi request reprint The role of LIP5 and CHMP5 in multivesicular body formation and HIV-1 budding in mammalian cells
    Diane McVey Ward
    University of Utah Health Sciences Center, Department of Pathology, Salt Lake City, Utah 84132, USA
    J Biol Chem 280:10548-55. 2005
    ....
  11. pmc Two distinct modes of ESCRT-III recognition are required for VPS4 functions in lysosomal protein targeting and HIV-1 budding
    Collin Kieffer
    Department of Biochemistry, University of Utah School of Medicine, Salt Lake City, UT 84112 5650, USA
    Dev Cell 15:62-73. 2008
    ....
  12. pmc Yap5 is an iron-responsive transcriptional activator that regulates vacuolar iron storage in yeast
    Liangtao Li
    Department of Pathology, University of Utah School of Medicine, Salt Lake City, UT 84132, USA
    Mol Cell Biol 28:1326-37. 2008
    ..Iron affects the sulfhydryl status of Yap5, which is indicative of the generation of intramolecular disulfide bonds. These results show that Yap5 is an iron-sensing transcription factor and that iron regulates transcriptional activation...
  13. pmc Genetic dissection of a mitochondria-vacuole signaling pathway in yeast reveals a link between chronic oxidative stress and vacuolar iron transport
    Liangtao Li
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    J Biol Chem 285:10232-42. 2010
    ..These results suggest that mitochondria-induced oxidant damage is responsible for activating Ccc1 and that Fra1 and Tsa1 can reduce oxidant damage...
  14. pmc Genetic and biochemical analysis of high iron toxicity in yeast: iron toxicity is due to the accumulation of cytosolic iron and occurs under both aerobic and anaerobic conditions
    Huilan Lin
    Department of Pharmacology, School of Medicine, University of California at San Diego, La Jolla, California 92093, USA
    J Biol Chem 286:3851-62. 2011
    ..Deletion of TSA1, which encodes a peroxiredoxin, exacerbated iron toxicity in Δccc1 cells under both aerobic and anaerobic conditions, suggesting a unique role for Tsa1 in iron toxicity...
  15. pmc The role of ubiquitination in hepcidin-independent and hepcidin-dependent degradation of ferroportin
    Ivana De Domenico
    Department of Internal Medicine, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Cell Metab 14:635-46. 2011
    ....
  16. pmc Induction of FPN1 transcription by MTF-1 reveals a role for ferroportin in transition metal efflux
    Marie Berengere Troadec
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT, USA
    Blood 116:4657-64. 2010
    ..We demonstrate that Fpn can transport zinc and can protect zinc sensitive cells from high zinc toxicity...
  17. pmc Hepcidin mediates transcriptional changes that modulate acute cytokine-induced inflammatory responses in mice
    Ivana De Domenico
    Department of Internal Medicine, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    J Clin Invest 120:2395-405. 2010
    ..The results of our study suggest a new function for hepcidin in modulating acute inflammatory responses...
  18. ncbi request reprint Transcription of the yeast iron regulon does not respond directly to iron but rather to iron-sulfur cluster biosynthesis
    Opal S Chen
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    J Biol Chem 279:29513-8. 2004
    ..Regulation of transcription by Fe-S biosynthesis represents a mechanism by which cellular iron acquisition is integrated with mitochondrial iron metabolism...
  19. ncbi request reprint A mitochondrial-vacuolar signaling pathway in yeast that affects iron and copper metabolism
    Liangtao Li
    Division of Immunology and Cell Biology, Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    J Biol Chem 279:33653-61. 2004
    ..These results suggest that deletion of mitochondrial proteins can alter vacuolar metal homeostasis. The data also indicate that increased expression of the AFT1-regulated gene(s) can disrupt copper homeostasis...
  20. pmc The hepcidin-binding site on ferroportin is evolutionarily conserved
    Ivana De Domenico
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Cell Metab 8:146-56. 2008
    ..The affinity of hepcidin for the HBD permits a rapid, sensitive assay of hepcidin from all species and yields insights into the evolution of hepcidin...
  21. pmc Yap5 protein-regulated transcription of the TYW1 gene protects yeast from high iron toxicity
    Liangtao Li
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    J Biol Chem 286:38488-97. 2011
    ..We conclude that the Yap5-mediated induction of TYW1 provides protection from high iron toxicity by the consumption of free cytosolic iron through the formation of protein-bound iron-sulfur clusters...
  22. ncbi request reprint PKR1 encodes an assembly factor for the yeast V-type ATPase
    Sandra R Davis-Kaplan
    Division of Immunology and Cell Biology, Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132 2501, USA
    J Biol Chem 281:32025-35. 2006
    ..Our data indicate that Pkr1p functions together with the other V-ATPase assembly factors in the ER to efficiently assemble the V-ATPase membrane sector...
  23. pmc Recruitment of Tup1p and Cti6p regulates heme-deficient expression of Aft1p target genes
    Robert J Crisp
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132 2501, USA
    EMBO J 25:512-21. 2006
    ..We hypothesize that transcription of the siderophore transporter ARN1 permits yeast to accumulate iron in the absence of oxygen and to deny iron to competing organisms...
  24. ncbi request reprint Inhibition of heme biosynthesis prevents transcription of iron uptake genes in yeast
    Robert J Crisp
    Division of Cell Biology and Immunology, Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah, 84132, USA
    J Biol Chem 278:45499-506. 2003
    ..Finally, we provide evidence that under heme-depleted conditions, yeast are able to regulate mitochondrial iron uptake and do not accumulate pathologic iron concentrations, as is seen when iron-sulfur cluster synthesis is disrupted...
  25. pmc A role for iron-sulfur clusters in the regulation of transcription factor Yap5-dependent high iron transcriptional responses in yeast
    Liangtao Li
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    J Biol Chem 287:35709-21. 2012
    ..These results suggest that the high iron transcriptional response, as well as the low iron transcriptional response, senses iron-sulfur clusters...
  26. pmc Mon1a protein acts in trafficking through the secretory apparatus
    Dustin C Bagley
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    J Biol Chem 287:25577-88. 2012
    ..These data provide strong evidence for a role for Mon1a in anterograde trafficking through the secretory apparatus...
  27. ncbi request reprint Gain-of-function mutations identify amino acids within transmembrane domains of the yeast vacuolar transporter Zrc1 that determine metal specificity
    Huilan Lin
    Department of Pathology, Department of Internal Medicine, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Biochem J 422:273-83. 2009
    ..These results suggest that substrate selection involves co-operativity between transmembrane domains...
  28. pmc Decoupling ferritin synthesis from free cytosolic iron results in ferritin secretion
    Ivana De Domenico
    Department of Internal Medicine, School of Medicine, University of Utah, Salt Lake City, UT 84132 2501, USA
    Cell Metab 13:57-67. 2011
    ..Secretion of ferritin chains provides a mechanism that limits ferritin nanocage assembly and ferritin-mediated iron sequestration in the absence of the translational inhibition of ferritin synthesis...
  29. pmc Ferroportin-mediated mobilization of ferritin iron precedes ferritin degradation by the proteasome
    Ivana De Domenico
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    EMBO J 25:5396-404. 2006
    ..Thus, ferritin degradation can occur through two different mechanisms...
  30. pmc Regulation of mitochondrial iron import through differential turnover of mitoferrin 1 and mitoferrin 2
    Prasad N Paradkar
    Department of Pathology, University of Utah School of Medicine, Salt Lake City, Utah 84132, USA
    Mol Cell Biol 29:1007-16. 2009
    ..These results suggest that mitochondrial iron accumulation is tightly regulated and that controlling mitoferrin levels within the mitochondrial membrane provides a mechanism to regulate mitochondrial iron levels...
  31. ncbi request reprint YKE4 (YIL023C) encodes a bidirectional zinc transporter in the endoplasmic reticulum of Saccharomyces cerevisiae
    Attila Kumanovics
    Division of Cell Biology and Immunology, Department of Pathology, University of Utah School of Medicine, Salt Lake City, Utah 84132, USA
    J Biol Chem 281:22566-74. 2006
    ....
  32. pmc Targeted deletion of the mouse Mitoferrin1 gene: from anemia to protoporphyria
    Marie Berengere Troadec
    Department of Pathology, School of Medicine, University of Utah, 50 North Medical Drive, Salt Lake City, UT 84132, USA
    Blood 117:5494-502. 2011
    ..Our results show that the activity of mitoferrin1 is required to manage an increase in heme synthesis. The data also show that alterations in heme synthesis within hepatocytes can lead to protoporphyria and hepatotoxicity...
  33. pmc Hepcidin-induced internalization of ferroportin requires binding and cooperative interaction with Jak2
    Ivana De Domenico
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132 2501, USA
    Proc Natl Acad Sci U S A 106:3800-5. 2009
    ..These results provide a molecular explanation for the dominant inheritance of hepcidin resistant iron overload disease...
  34. pmc Human mutation D157G in ferroportin leads to hepcidin-independent binding of Jak2 and ferroportin down-regulation
    Ivana De Domenico
    Division of Hematology, Department of Medicine, School of Medicine, University of Utah, Salt Lake City, USA
    Blood 115:2956-9. 2010
    ..These results identify a hepcidin-independent regulation of Fpn that can result in alterations in iron homeostasis...
  35. pmc Iron depletion limits intracellular bacterial growth in macrophages
    Prasad N Paradkar
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    Blood 112:866-74. 2008
    ....
  36. pmc Genetic analysis of iron citrate toxicity in yeast: implications for mammalian iron homeostasis
    Opal S Chen
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Proc Natl Acad Sci U S A 99:16922-7. 2002
    ..These results suggest that high levels of citrate may promote iron-mediated tissue damage...
  37. pmc Ferroportin-mediated iron transport: expression and regulation
    Diane M Ward
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    Biochim Biophys Acta 1823:1426-33. 2012
    ..Regulation of ferroportin is critical for iron homeostasis as alterations in ferroportin may result in either iron deficiency or iron overload. This article is part of a Special Issue entitled: Cell Biology of Metals...
  38. pmc The enlarged lysosomes in beige j cells result from decreased lysosome fission and not increased lysosome fusion
    Nina Durchfort
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Traffic 13:108-19. 2012
    ..We further show that overexpression of the Chs1/Lyst protein gives rise to a faster rate of lysosome fission. These results indicate that Chs1/Lyst regulates lysosome size by affecting fission...
  39. pmc Toll-like receptors mediate induction of hepcidin in mice infected with Borrelia burgdorferi
    Curry L Koening
    Division of Rheumatology, Department of Internal Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Blood 114:1913-8. 2009
    ..Hepcidin expression induced by B burgdorferi was mediated primarily by activation of Toll-like receptor 2...
  40. pmc Specific iron chelators determine the route of ferritin degradation
    Ivana De Domenico
    Department of Internal Medicine, University of Utah School of Medicine, Salt Lake City, UT 84132, USA
    Blood 114:4546-51. 2009
    ..These results indicate that ferritin degradation occurs by 2 routes: a DFO-induced entry of ferritin into lysosomes and a cytosolic route in which iron is extracted from ferritin before degradation by the proteasome...
  41. ncbi request reprint Bph1p, the Saccharomyces cerevisiae homologue of CHS1/beige, functions in cell wall formation and protein sorting
    Shelly L Shiflett
    Department of Pathology, Division of Cell Biology and Immunology, University of Utah Health Science Center, Salt Lake City, Utah 84132, USA
    Traffic 5:700-10. 2004
    ..Together, these data suggest that Bph1p associates with a membrane and is involved in protein sorting and cell wall formation...
  42. pmc Functional studies of hephaestin in yeast: evidence for multicopper oxidase activity in the endocytic pathway
    Liangtao Li
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Biochem J 375:793-8. 2003
    ....
  43. doi request reprint A Tf-independent iron transport system required for organogenesis
    Marie Berengere Troadec
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Dev Cell 16:3-4. 2009
    ..in this issue of Developmental Cell show that ferritin, through its binding to the cell surface Scara5 receptor, can also deliver iron to cells, permitting kidney organogenesis...
  44. ncbi request reprint Chediak-Higashi syndrome
    Jerry Kaplan
    Department of Pathology, University of Utah School of Medicine, 30 N 1900 E, Salt Lake City, UT 84132, USA
    Curr Opin Hematol 15:22-9. 2008
    ..This review will discuss the advances made in understanding the clinical aspects of the syndrome and the function of CHS1/LYST/Beige...
  45. ncbi request reprint Genome-wide analysis of iron-dependent growth reveals a novel yeast gene required for vacuolar acidification
    Sandra R Davis-Kaplan
    Division of Immunology and Cell Biology, Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    J Biol Chem 279:4322-9. 2004
    ..This gene contains two introns, has homologues in other Saccharomyces strains, and shows weak homology to a component of the vacuolar H+-ATPase found in organisms as diverse as insect and cow...
  46. ncbi request reprint Characterization of Vta1p, a class E Vps protein in Saccharomyces cerevisiae
    Shelly L Shiflett
    Department of Pathology, Division of Cell Biology and Immunology, University of Utah Health Science Center, Salt Lake City, Utah 84132 2501, USA
    J Biol Chem 279:10982-90. 2004
    ..Membrane-associated Vta1p co-purified with Vps60p, suggesting that Vta1p is a class E Vps protein that interacts with Vps60p on a prevacuolar compartment...
  47. pmc Ferroxidase activity is required for the stability of cell surface ferroportin in cells expressing GPI-ceruloplasmin
    Ivana De Domenico
    Department of Pathology, School of Medicine, University of Utah, 50 North Medical Drive, Salt Lake City, UT 84132, USA
    EMBO J 26:2823-31. 2007
    ..The requirement for a ferroxidase to maintain iron transport activity represents a new mechanism of regulating cellular iron export, a new function for Cp and an explanation for brain iron overload in patients with aceruloplasminemia...
  48. pmc Hepcidin and ferroportin: the new players in iron metabolism
    Ivana De Domenico
    Department of Internal Medicine, School of Medicine, University of Utah, Salt Lake City, Utah, USA
    Semin Liver Dis 31:272-9. 2011
    ..Ferroportin degradation can occur by hepcidin-dependent and hepcidin-independent internalization. Ferroportin expression is regulated transcriptionally and posttranslationally...
  49. ncbi request reprint A dominant allele of PDR1 alters transition metal resistance in yeast
    Marie Spencer Tuttle
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City 84132, USA
    J Biol Chem 278:1273-80. 2003
    ..Cells expressing PDR1(R821H) also showed increased resistance to copper and manganese because of increased metal export. These results suggest that expression of PDR1-regulated genes affects both efflux and storage of transition metals...
  50. pmc Hepcidin regulation: ironing out the details
    Ivana De Domenico
    Department of Pathology, University of Utah School of Medicine, Salt Lake City, UT 84106, USA
    J Clin Invest 117:1755-8. 2007
    ..These results demonstrate that recombinant HJV may be a useful therapeutic agent for treatment of the anemia of chronic disease, a disorder resulting from high levels of hepcidin expression...
  51. ncbi request reprint The protein network of HIV budding
    Uta K von Schwedler
    Department of Biochemistry, University of Utah, Salt Lake City, UT 84132, USA
    Cell 114:701-13. 2003
    ..These studies define a protein network required for human MVB biogenesis and indicate that the entire network participates in the release of HIV and probably many other viruses...
  52. pmc Evidence for the multimeric structure of ferroportin
    Ivana De Domenico
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Blood 109:2205-9. 2007
    ..These results support the hypothesis that the dominant inheritance of Fpn-iron overload disease is due to the dominant-negative effects of mutant Fpn proteins...
  53. ncbi request reprint Regulation of iron acquisition and storage: consequences for iron-linked disorders
    Ivana De Domenico
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    Nat Rev Mol Cell Biol 9:72-81. 2008
    ..Discoveries of iron transporters and insights into their regulation have provided important information about iron metabolism and genetic iron disorders...
  54. pmc Zebrafish as a model for defining the functional impact of mammalian ferroportin mutations
    Ivana De Domenico
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Blood 110:3780-3. 2007
    ..Expression of wild-type ferroportin or hepcidin-resistant ferroportin (N144H) does not affect erythropoiesis. Zebrafish provides a facile way of identifying which ferroportin mutants may lead to macrophage iron loading...
  55. doi request reprint A new wrinkle in the fold: hepcidin links inflammation to the unfolded protein response
    Ivana De Domenico
    Department of Internal Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Cell Metab 10:245-6. 2009
    ..Two recent papers (Oliveira et al., 2009; Vecchi et al., 2009) show that the UPR modulates transcription of the hormone hepcidin, which controls plasma iron levels and perhaps innate immunity...
  56. ncbi request reprint Expression of the Yeast Cation Diffusion Facilitators Mmt1 and Mmt2 Affects Mitochondrial and Cellular Iron Homeostasis: EVIDENCE FOR MITOCHONDRIAL IRON EXPORT
    Liangtao Li
    From the Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132
    J Biol Chem 289:17132-41. 2014
    ..Overexpression of MMT1 resulted in increased cytosolic iron whereas deletion of MMT1/MMT2 led to decreased cytosolic iron. These results support the hypothesis that Mmt1/2 function as mitochondrial iron exporters. ..
  57. pmc Hepcidin is elevated in mice injected with Mycoplasma arthritidis
    Curry L Koening
    Division of Hematology, Department of Internal Medicine, University of Utah, School of Medicine, 30 North 1900 East, Salt Lake City, 84132, Utah, USA
    J Inflamm (Lond) 6:33. 2009
    ..Both mice had an increase in serum ferritin but a decrease in serum transferrin saturation. In conclusion, serum hepcidin regulation in C3H/HeJ mice does not appear to be solely dependent upon TLR4 or IL-6...
  58. pmc Inhibition of Fe-S cluster biosynthesis decreases mitochondrial iron export: evidence that Yfh1p affects Fe-S cluster synthesis
    Opal S Chen
    Division of Immunology and Cell Biology, Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Proc Natl Acad Sci U S A 99:12321-6. 2002
    ..These results demonstrate a direct role of Yfh1p in the formation of Fe-S clusters and indicate that mitochondrial iron export requires Fe-S cluster biosynthesis...
  59. ncbi request reprint Chediak-Higashi syndrome: a clinical and molecular view of a rare lysosomal storage disorder
    Diane McVey Ward
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City 84132, USA
    Curr Mol Med 2:469-77. 2002
    ..The presence of the BEACH and WD40 domains defines a family of genes that encode extremely large proteins...
  60. ncbi request reprint Strategy and tactics in the evolution of iron acquisition
    Jerry Kaplan
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132 2408, USA
    Semin Hematol 39:219-26. 2002
    ..Ferric iron (Fe(3+)) transport systems are highly specific for iron, and in vertebrates are used to target iron transport to specific tissues...
  61. ncbi request reprint Chediak-Higashi Syndrome: a rare disorder of lysosomes and lysosome related organelles
    Shelly L Shiflett
    Department of Pathology, University of Utah School of Medicine, Salt Lake City, Utah, USA
    Pigment Cell Res 15:251-7. 2002
    ..The identification of CHS1/Beige has defined a family of genes containing a common BEACH motif. The function of these proteins in vesicular trafficking remains unknown...
  62. doi request reprint Some vertebrates go with the GLO
    Marie Berengere Troadec
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Cell 132:921-2. 2008
    ..This substrate preference switch is mediated by the membrane protein stomatin and is an evolutionary adaptation to vitamin C deficiency...
  63. ncbi request reprint Transition metal transport in yeast
    Anthony Van Ho
    Departments of Internal Medicine University of Utah, School of Medicine, Salt Lake City 84132, USA
    Annu Rev Microbiol 56:237-61. 2002
    ..Results from studies on S. cerevisiae have been used to understand metal transport in other species of yeast as well as in higher eukaryotes...
  64. pmc Regulation of Ribonucleotide Reductase during Iron Limitation
    Alexandra Seguin
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
    Mol Cell 44:683-4. 2011
    ..In this issue of Molecular Cell, Sanvisens et al. (2011) report a new mechanism for regulation of yeast ribonucleotide reductase activity that occurs during iron deprivation...
  65. ncbi request reprint Use of expression constructs to dissect the functional domains of the CHS/beige protein: identification of multiple phenotypes
    Diane McVey Ward
    Department of Pathology, School of Medicine, University of Utah, Salt Lake City, Utah 84132, USA
    Traffic 4:403-15. 2003
    ..These results suggest that the Beige protein interacts with at least two different partners and that the Beige protein affects cellular events, such as nuclear PtdIns(4,5)P2 localization, in addition to lysosome size...
  66. ncbi request reprint Frataxin knockin mouse
    Carlos J Miranda
    Department of Medicine, Centre Hospitalier de l Universite de Montreal, Hopital Notre Dame, Pav de Seve Y5608, 1560 rue Sherbrooke Est, H2L 4M1, Montreal, QC, Canada
    FEBS Lett 512:291-7. 2002
    ..These mice were viable and did not develop anomalies of motor coordination, iron metabolism or response to iron loading. Repeats were meiotically and mitotically stable...
  67. ncbi request reprint Mitoferrin is essential for erythroid iron assimilation
    George C Shaw
    Division of Hematology, Department of Medicine, Brigham and Women s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    Nature 440:96-100. 2006
    ..Our data show that mfrn functions as the principal mitochondrial iron importer essential for haem biosynthesis in vertebrate erythroblasts...
  68. pmc The molecular basis of ferroportin-linked hemochromatosis
    Ivana De Domenico
    Dipartimento di Scienze Microbiologiche Genetiche e Molecolari, Universita di Messina, 98166 Messina, Italy
    Proc Natl Acad Sci U S A 102:8955-60. 2005
    ..The behavior of mutant Fpn in cell culture and the ability of mutant Fpn to act as a dominant negative explain the dominant inheritance of the disease as well as the different patient phenotypes...
  69. ncbi request reprint Post-transcriptional regulation of the yeast high affinity iron transport system
    M Rosa Felice
    Dipartimento di Scienze Microbiologiche Genetiche e Molecolari, Universita di Messina, Salita Sperone 31, I 98166 Villaggio S Agata, Messina I 98166, Italy
    J Biol Chem 280:22181-90. 2005
    ....
  70. ncbi request reprint Hepcidin regulates cellular iron efflux by binding to ferroportin and inducing its internalization
    Elizabeta Nemeth
    Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA, USA
    Science 306:2090-3. 2004
    ..The posttranslational regulation of ferroportin by hepcidin may thus complete a homeostatic loop: Iron regulates the secretion of hepcidin, which in turn controls the concentration of ferroportin on the cell surface...
  71. pmc The WD repeat protein FAN regulates lysosome size independent from abnormal downregulation/membrane recruitment of protein kinase C
    Heike Möhlig
    Institut fur Immunologie, Universitätsklinikum Schleswig Holstein Campus Kiel, Michaelisstr 5, 24105 Kiel, Germany
    Exp Cell Res 313:2703-18. 2007
    ..In summary, FAN mediates activation of N-SMase as well as regulation of lysosome size by signaling pathways that operate independent from activation/membrane recruitment of PKC...
  72. ncbi request reprint Localization of iron in Arabidopsis seed requires the vacuolar membrane transporter VIT1
    Sun A Kim
    Department of Biological Sciences, Dartmouth College, Hanover, NH 03755, USA
    Science 314:1295-8. 2006
    ..We have uncovered a fundamental aspect of seed biology that will ultimately aid the development of nutrient-rich seed, benefiting both human health and agricultural productivity...
  73. doi request reprint A heme export protein is required for red blood cell differentiation and iron homeostasis
    Siobán B Keel
    Division of Hematology, University of Washington, Seattle, WA 98195, USA
    Science 319:825-8. 2008
    ..Thus, the trafficking of heme, and not just elemental iron, facilitates erythropoiesis and systemic iron balance...
  74. ncbi request reprint Grey, a novel mutation in the murine Lyst gene, causes the beige phenotype by skipping of exon 25
    Fabian Runkel
    Anatomisches Institut, Universitat Bonn, Germany
    Mamm Genome 17:203-10. 2006
    ....
  75. ncbi request reprint Genetic variation in Mon1a affects protein trafficking and modifies macrophage iron loading in mice
    Fudi Wang
    Division of Hematology Oncology, Children s Hospital Boston, Boston, Massachusetts, 02115 USA
    Nat Genet 39:1025-32. 2007
    ..Mon1a is also important for trafficking of cell-surface and secreted molecules unrelated to iron metabolism, suggesting that it has a fundamental role in the mammalian secretory apparatus...
  76. pmc The N-terminus of hepcidin is essential for its interaction with ferroportin: structure-function study
    Elizabeta Nemeth
    Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095 1690, USA
    Blood 107:328-33. 2006
    ..G71D and K83R, substitutions previously described in humans, did not affect hepcidin activity. Apart from the essential nature of the N-terminus, hepcidin structure appears permissive for mutations...
  77. pmc Molecular and clinical correlates in iron overload associated with mutations in ferroportin
    Ivana De Domenico
    Dipartimento di Scienze Microbiologiche Genetiche e Molecolari, Universita di Messina, 98166 Messina, Italia
    Haematologica 91:1092-5. 2006
    ..We suggest that FpnG80S represents a class of Fpn mutants whose behavior in vitro does not explain the patients' phenotype...
  78. pmc Iron overload due to mutations in ferroportin
    Ivana De Domenico
    Dipartimento di Scienze Microbiologiche Genetiche e Molecolari, Universita di Messina, Messina, Italy
    Haematologica 91:92-5. 2006
    ....
  79. ncbi request reprint Deficiency of glutaredoxin 5 reveals Fe-S clusters are required for vertebrate haem synthesis
    Rebecca A Wingert
    Stem Cell Program and Division Hematology Oncology Children s Hospital and Dana Farber Cancer Institute, Howard Hughes Medical Institute, Harvard Medical School, Boston, Massachusetts 02115, USA
    Nature 436:1035-39. 2005
    ..These findings uncover a connection between haem biosynthesis and Fe-S clusters, indicating that haemoglobin production in the differentiating red cell is regulated through Fe-S cluster assembly...
  80. ncbi request reprint Regulatory oversight of the iron trade: posttranscriptional regulation in yeast
    Craig D Kaplan
    Department of Structural Biology, Stanford University, Palo Alto, CA 94305, USA
    Cell Metab 2:4-6. 2005
    ..These studies demonstrate sophisticated systems that limit iron uptake under conditions of abundance and lead to metabolic remodeling when iron is limited...
  81. pmc Defective lysosomal exocytosis and plasma membrane repair in Chediak-Higashi/beige cells
    Chau Huynh
    Section of Microbial Pathogenesis and Department of Cell Biology, Yale University School of Medicine, 295 Congress Avenue, New Haven, CT 06536, USA
    Proc Natl Acad Sci U S A 101:16795-800. 2004
    ..Thus, the severe symptoms exhibited by CHS patients may also include defects in the ability of cells to repair plasma membrane lesions...
  82. ncbi request reprint A fungal multicopper oxidase restores iron homeostasis in aceruloplasminemia
    Z Leah Harris
    Department of Anesthesiology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA
    Blood 103:4672-3. 2004
    ..These results show the conservation of function of copper-containing proteins in eukaryotic iron metabolism...
  83. pmc The flatiron mutation in mouse ferroportin acts as a dominant negative to cause ferroportin disease
    Irene E Zohn
    Howard Hughes Medical Institute, Department of Pediatrics, Section of Developmental Biology, University of Colorado at Denver and Health Sciences Center, Aurora
    Blood 109:4174-80. 2007
    ..These results demonstrate that mutations in Fpn resulting in protein mislocalization act in a dominant-negative fashion to cause disease, and the Fpn(ffe) mouse represents the first mouse model of ferroportin disease...

Research Grants64

  1. Mechanism and Regulation of Iron Export by Ferroportin
    Jerry Kaplan; Fiscal Year: 2010
    ..Our studies will provide information on how alterations in ferroportin result in disease and create new opportunities to manage and diagnose human diseases due to altered iron metabolism. ..
  2. GENETIC ANALYSIS OF MITOCHONDRIAL IRON METABOLISM
    Jerry Kaplan; Fiscal Year: 2007
    ..We propose to determine the mechanism by which the absence of heme affects iron transport. ..
  3. Mechanism and Regulation of Iron Export by Ferroportin
    Jerry Kaplan; Fiscal Year: 2006
    ..These studies will probe the mechanism that underlines the regulation of iron export by hepcidin and will define the roles of hepcidin and Fpn in iron physiology. ..
  4. GENETIC ANALYSIS OF MITOCHONDRIAL IRON METABOLISM
    Jerry Kaplan; Fiscal Year: 2006
    ..We propose to determine the mechanism by which the absence of heme affects iron transport. ..
  5. BIOIRON RESEARCH CONFERENCE 2005
    Jerry Kaplan; Fiscal Year: 2005
    ..The overall goal of the Bioiron (2005) World Congress is the international dissemination of important new research results to investigators working in the field of iron metabolism. ..
  6. GENETIC ANALYSIS OF MITOCHONDRIAL IRON METABOLISM
    Jerry Kaplan; Fiscal Year: 2005
    ..We propose to determine the mechanism by which the absence of heme affects iron transport. ..
  7. GENETIC ANALYSIS OF MITOCHONDRIAL IRON METABOLISM
    Jerry Kaplan; Fiscal Year: 2004
    ..We propose to determine the mechanism by which the absence of heme affects iron transport. ..
  8. GENETIC ANALYSIS OF MITOCHONDRIAL IRON METABOLISM
    Jerry Kaplan; Fiscal Year: 2003
    ..We propose to determine the mechanism by which the absence of heme affects iron transport. ..
  9. GENETIC ANALYSIS OF MITOCHONDRIAL IRON METAB0LISM
    Jerry Kaplan; Fiscal Year: 2002
    ..They will determine if defective heme biosynthesis results, as it does in reticulocytes, in excessive iron deposition in mitochondria. These studies will help define the regulation of mitochondrial iron metabolism. ..
  10. GENETIC ANALYSIS OF MITOCHONDRIAL IRON METAB0LISM
    Jerry Kaplan; Fiscal Year: 2001
    ..They will determine if defective heme biosynthesis results, as it does in reticulocytes, in excessive iron deposition in mitochondria. These studies will help define the regulation of mitochondrial iron metabolism. ..
  11. GENETIC ANALYSIS OF MITOCHONDRIAL IRON METAB0LISM
    Jerry Kaplan; Fiscal Year: 2000
    ..They will determine if defective heme biosynthesis results, as it does in reticulocytes, in excessive iron deposition in mitochondria. These studies will help define the regulation of mitochondrial iron metabolism. ..
  12. GENETIC ANALYSIS OF MITOCHONDRIAL IRON METAB0LISM
    Jerry Kaplan; Fiscal Year: 2000
    ..They will determine if defective heme biosynthesis results, as it does in reticulocytes, in excessive iron deposition in mitochondria. These studies will help define the regulation of mitochondrial iron metabolism. ..
  13. Automated Confocal Instrument for Core Imaging Facility
    Jerry Kaplan; Fiscal Year: 2007
    ....
  14. Mechanism and Regulation of Iron Export by Ferroportin
    Jerry Kaplan; Fiscal Year: 2007
    ..These studies will probe the mechanism that underlines the regulation of iron export by hepcidin and will define the roles of hepcidin and Fpn in iron physiology. ..
  15. Mechanism and Regulation of Iron Export by Ferroportin
    Jerry Kaplan; Fiscal Year: 2009
    ..These studies will probe the mechanism that underlines the regulation of iron export by hepcidin and will define the roles of hepcidin and Fpn in iron physiology. ..
  16. RECEPTOR MEDIATED ENDOCYTOSIS IN ALVEOLAR MACROPHAGES
    Jerry Kaplan; Fiscal Year: 2010
    ..Our studies on macrophage functions will provide information that may be used to manage and diagnose human diseases that affect vesicular trafficking and lung homeostasis. ..
  17. FACTORS REGULATING THE CELLULAR UPTAKE OF IRON
    Jerry Kaplan; Fiscal Year: 2010
    ..We also propose to identify mechanisms that regulate iron storage. Our studies will provide information that may be used to manage and diagnose human diseases to altered iron metabolism. ..
  18. GENETIC ANALYSIS OF MITOCHONDRIAL IRON METABOLISM
    Jerry Kaplan; Fiscal Year: 2010
    ....
  19. RECEPTOR MEDIATED ENDOCYTOSIS IN ALVEOLAR MACROPHAGES
    Jerry Kaplan; Fiscal Year: 2009
    ..Our studies on macrophage functions will provide information that may be used to manage and diagnose human diseases that affect vesicular trafficking and lung homeostasis. ..
  20. GENETIC ANALYSIS OF MITOCHONDRIAL IRON METABOLISM
    Jerry Kaplan; Fiscal Year: 2009
    ....
  21. BioIron Research Conference 2007
    Jerry Kaplan; Fiscal Year: 2007
    ..The overall goal of the BioIron 2007 World Congress is the international dissemination of important new research results to investigators working in the field of iron metabolism. ..
  22. Short-Term Training: Students in Health Professional Sc
    Jerry Kaplan; Fiscal Year: 2007
    ..We request funding for 25 fellowships per year. This number is based on the past number of applicants and on further training for highly meritorious applicants. ..
  23. FACTORS REGULATING THE CELLULAR UPTAKE OF IRON
    Jerry Kaplan; Fiscal Year: 1990
    ..These studies will yield information on the mechanisms of tissue iron overload and the role of iron in normal and pathological processes...
  24. GENETIC ANALYSIS OF MITOCHONDRIAL IRON METAB0LISM
    Jerry Kaplan; Fiscal Year: 1999
    ..They will determine if defective heme biosynthesis results, as it does in reticulocytes, in excessive iron deposition in mitochondria. These studies will help define the regulation of mitochondrial iron metabolism. ..
  25. FACTORS REGULATING THE CELLULAR UPTAKE OF IRON
    Jerry Kaplan; Fiscal Year: 1999
    ..Our studies will clarify the mechanisms that regulate cellular iron content and the role of iron in normal and pathological processes. ..
  26. RECEPTOR MEDIATED ENDOCYTOSIS IN ALVEOLAR MACROPHAGES
    Jerry Kaplan; Fiscal Year: 1993
    ..These studies will yield insight into the normal mechanisms of macrophage activities and into derangements which may compromise lung function...
  27. RECEPTOR MEDIATED ENDOCYTOSIS IN ALVEOLAR MACROPHAGES
    Jerry Kaplan; Fiscal Year: 1991
    ..This latter study will yield insights into the mechanism of pulmonary hemosiderosis. The studies will increase our understanding of the mechanisms of alveolar macrophage membrane dynamics and the role of macrophages in lung disease...
  28. RECEPTOR MEDIATED ENDOCYTOSIS IN ALVEOLAR MACROPHAGES
    Jerry Kaplan; Fiscal Year: 1990
    ..This latter study will yield insights into the mechanism of pulmonary hemosiderosis. The studies will increase our understanding of the mechanisms of alveolar macrophage membrane dynamics and the role of macrophages in lung disease...
  29. Mechanism and Regulation of Iron Export by Ferroportin
    Jerry Kaplan; Fiscal Year: 2005
    ..These studies will probe the mechanism that underlines the regulation of iron export by hepcidin and will define the roles of hepcidin and Fpn in iron physiology. ..
  30. RECEPTOR MEDIATED ENDOCYTOSIS IN ALVEOLAR MACROPHAGES
    Jerry Kaplan; Fiscal Year: 1992
    ..These studies will yield insight into the normal mechanisms of macrophage activities and into derangements which may compromise lung function...
  31. FACTORS REGULATING THE CELLULAR UPTAKE OF IRON
    Jerry Kaplan; Fiscal Year: 1993
    ..These studies will yield information on the mechanisms of tissue iron overload and the role of iron in normal and pathological processes...
  32. FACTORS REGULATING THE CELLULAR UPTAKE OF IRON
    Jerry Kaplan; Fiscal Year: 1992
    ..These studies will yield information on the mechanisms of tissue iron overload and the role of iron in normal and pathological processes...
  33. FACTORS REGULATING THE CELLULAR UPTAKE OF IRON
    Jerry Kaplan; Fiscal Year: 1991
    ..These studies will yield information on the mechanisms of tissue iron overload and the role of iron in normal and pathological processes...
  34. Mechanism and Regulation of Iron Export by Ferroportin
    Jerry Kaplan; Fiscal Year: 2009
    ..These studies will probe the mechanism that underlines the regulation of iron export by hepcidin and will define the roles of hepcidin and Fpn in iron physiology. ..