LAURIE SIMON KAGUNI

Summary

Affiliation: Michigan State University
Country: USA

Publications

  1. pmc Clustering of Alpers disease mutations and catalytic defects in biochemical variants reveal new features of molecular mechanism of the human mitochondrial replicase, Pol γ
    Liliya Euro
    Research Programs Unit, Molecular Neurology, Biomedicum Helsinki, University of Helsinki, Haartmaninkatu 8, 00290 Helsinki, Finland
    Nucleic Acids Res 39:9072-84. 2011
  2. pmc Functional roles of the N- and C-terminal regions of the human mitochondrial single-stranded DNA-binding protein
    Marcos T Oliveira
    Department of Biochemistry and Molecular Biology, Center for Mitochondrial Science and Medicine, and Graduate Program in Genetics, Michigan State University, East Lansing, Michigan, United States of America
    PLoS ONE 5:e15379. 2010
  3. ncbi request reprint DNA polymerase gamma, the mitochondrial replicase
    Laurie S Kaguni
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, Michigan 48824 1319, USA
    Annu Rev Biochem 73:293-320. 2004
  4. ncbi request reprint Purification strategies for Drosophila mtDNA replication proteins in native and recombinant form. DNA polymerase gamma
    Carol L Farr
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, MI, USA
    Methods Mol Biol 197:273-83. 2002
  5. ncbi request reprint Purification strategies for Drosophila mtDNA replication proteins in native and recombinant form. Mitochondrial single-stranded DNA-binding protein
    Carol L Farr
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, MI, USA
    Methods Mol Biol 197:285-94. 2002
  6. pmc Functional importance of the conserved N-terminal domain of the mitochondrial replicative DNA helicase
    Yuichi Matsushima
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing MI 48824 1319, USA
    Biochim Biophys Acta 1787:290-5. 2009
  7. pmc Animal models of mitochondrial DNA transactions in disease and ageing
    Marcos T Oliveira
    Department of Biochemistry and Molecular Biology, Center for Mitochondrial Science and Medicine, and Graduate Program in Genetics, Michigan State University, East Lansing, MI 48824 1319, USA
    Exp Gerontol 45:489-502. 2010
  8. pmc Modular architecture of the hexameric human mitochondrial DNA helicase
    Tawn D Ziebarth
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, MI 48823, USA
    J Mol Biol 367:1382-91. 2007
  9. doi request reprint Comparative purification strategies for Drosophila and human mitochondrial DNA replication proteins: DNA polymerase gamma and mitochondrial single-stranded DNA-binding protein
    Marcos T Oliveira
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, MI, USA
    Methods Mol Biol 554:37-58. 2009
  10. ncbi request reprint Physiological and biochemical defects in functional interactions of mitochondrial DNA polymerase and DNA-binding mutants of single-stranded DNA-binding protein
    Carol L Farr
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, Michigan 48824 1319, USA
    J Biol Chem 279:17047-53. 2004

Collaborators

  • Anu Suomalainen
  • Kyle E Miller
  • Francisco Martinez-Azorin
  • Yuichi Matsushima
  • Carol L Farr
  • Tawn D Ziebarth
  • Marcos T Oliveira
  • Rafael Garesse
  • Ningguang Luo
  • Miguel Angel Fernández-Moreno
  • Cristina Adán
  • Magdalena M Makowska-Grzyska
  • Li Fan
  • Liliya Euro
  • Rehan M Baqri
  • Rosana Hernández-Sierra
  • Alvaro Sánchez-Martínez
  • Petri T Luoma
  • Balaji Iyengar
  • Gregory A Farnum
  • Eino Palin
  • Rafael Núñez-Ramírez
  • Jose Maria Carazo
  • Rocio Gonzalez-Soltero
  • Yu ichi Goto
  • Brittany A Turner
  • Bradley D Hammond
  • Mary B Rheuben
  • Raquel Marco-Ferreres
  • Emiliano Gonzalez-Vioque
  • Manuel Calleja
  • Juan J Aragon
  • John A Tainer
  • Sangbumn Kim
  • Kevin T Schaefer
  • Paula Clemente
  • Pilar Ochoa
  • Kathleen M Randolph
  • Wolfgang N Löscher
  • Rita Horvath
  • Julia Wanschitz
  • Stefan Kiechl
  • Anthony T Lagina
  • Ana Regina Campos

Detail Information

Publications27

  1. pmc Clustering of Alpers disease mutations and catalytic defects in biochemical variants reveal new features of molecular mechanism of the human mitochondrial replicase, Pol γ
    Liliya Euro
    Research Programs Unit, Molecular Neurology, Biomedicum Helsinki, University of Helsinki, Haartmaninkatu 8, 00290 Helsinki, Finland
    Nucleic Acids Res 39:9072-84. 2011
    ..Our results suggest that cluster prediction can be used as a diagnosis-supporting tool to evaluate the pathogenic role of new Pol γ variants...
  2. pmc Functional roles of the N- and C-terminal regions of the human mitochondrial single-stranded DNA-binding protein
    Marcos T Oliveira
    Department of Biochemistry and Molecular Biology, Center for Mitochondrial Science and Medicine, and Graduate Program in Genetics, Michigan State University, East Lansing, Michigan, United States of America
    PLoS ONE 5:e15379. 2010
    ..We discuss our findings in the context of species-specific components of the mtDNA replisome, and in comparison with various prokaryotic DNA replication machineries...
  3. ncbi request reprint DNA polymerase gamma, the mitochondrial replicase
    Laurie S Kaguni
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, Michigan 48824 1319, USA
    Annu Rev Biochem 73:293-320. 2004
    ..This review describes our current understanding of pol gamma biochemistry and biology, and it introduces other key proteins that function at the mitochondrial DNA replication fork...
  4. ncbi request reprint Purification strategies for Drosophila mtDNA replication proteins in native and recombinant form. DNA polymerase gamma
    Carol L Farr
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, MI, USA
    Methods Mol Biol 197:273-83. 2002
  5. ncbi request reprint Purification strategies for Drosophila mtDNA replication proteins in native and recombinant form. Mitochondrial single-stranded DNA-binding protein
    Carol L Farr
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, MI, USA
    Methods Mol Biol 197:285-94. 2002
  6. pmc Functional importance of the conserved N-terminal domain of the mitochondrial replicative DNA helicase
    Yuichi Matsushima
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing MI 48824 1319, USA
    Biochim Biophys Acta 1787:290-5. 2009
    ....
  7. pmc Animal models of mitochondrial DNA transactions in disease and ageing
    Marcos T Oliveira
    Department of Biochemistry and Molecular Biology, Center for Mitochondrial Science and Medicine, and Graduate Program in Genetics, Michigan State University, East Lansing, MI 48824 1319, USA
    Exp Gerontol 45:489-502. 2010
    ....
  8. pmc Modular architecture of the hexameric human mitochondrial DNA helicase
    Tawn D Ziebarth
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, MI 48823, USA
    J Mol Biol 367:1382-91. 2007
    ..Our findings place the mitochondrial DNA helicase firmly in the DnaB-like family of replicative DNA helicases...
  9. doi request reprint Comparative purification strategies for Drosophila and human mitochondrial DNA replication proteins: DNA polymerase gamma and mitochondrial single-stranded DNA-binding protein
    Marcos T Oliveira
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, MI, USA
    Methods Mol Biol 554:37-58. 2009
    ..Their purification in homogeneous, active form represents the first step toward our long-term goal to understand their biochemistry, biology, and functions at the mitochondrial DNA replication fork...
  10. ncbi request reprint Physiological and biochemical defects in functional interactions of mitochondrial DNA polymerase and DNA-binding mutants of single-stranded DNA-binding protein
    Carol L Farr
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, Michigan 48824 1319, USA
    J Biol Chem 279:17047-53. 2004
    ....
  11. ncbi request reprint Drosophila mitochondrial transcription factor B1 modulates mitochondrial translation but not transcription or DNA copy number in Schneider cells
    Yuichi Matsushima
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, Michigan 48824 1319, USA
    J Biol Chem 280:16815-20. 2005
    ..Our work represents the first study to document the role of mtTFB1 in vivo and establishes clearly functional differences between mtTFB1 and mtTFB2...
  12. ncbi request reprint Mutations in the spacer region of Drosophila mitochondrial DNA polymerase affect DNA binding, processivity, and the balance between Pol and Exo function
    Ningguang Luo
    Graduate Program in Genetics and Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, Michigan 48823, USA
    J Biol Chem 280:2491-7. 2005
    ....
  13. pmc Mitochondrial Lon protease regulates mitochondrial DNA copy number and transcription by selective degradation of mitochondrial transcription factor A (TFAM)
    Yuichi Matsushima
    Department of Biochemistry and Molecular Biology, and Center for Mitochondrial Science and Medicine, Michigan State University, East Lansing, MI 48824 1319, USA
    Proc Natl Acad Sci U S A 107:18410-5. 2010
    ..The increased TFAMmtDNA ratio in turn causes inhibition of mitochondrial transcription. We conclude that Lon regulates mitochondrial transcription by stabilizing the mitochondrial TFAMmtDNA ratio via selective degradation of TFAM...
  14. pmc Physiological and biochemical defects in carboxyl-terminal mutants of mitochondrial DNA helicase
    Yuichi Matsushima
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, MI 48824 1319, USA
    J Biol Chem 283:23964-71. 2008
    ..Our findings may be applicable to understand the role of the carboxyl-terminal region in superfamily 4 DNA helicases in general...
  15. doi request reprint Purification strategy for recombinant forms of the human mitochondrial DNA helicase
    Tawn D Ziebarth
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, MI, USA
    Methods Mol Biol 554:121-6. 2009
    ..Minimizing the number of steps and the time elapsed for purification of this enzyme facilitates studies of its structure and mechanism and allows elucidation of native features of both wild-type- and human disease-related forms...
  16. pmc Disruption of mitochondrial DNA replication in Drosophila increases mitochondrial fast axonal transport in vivo
    Rehan M Baqri
    Department of Zoology, Michigan State University, East Lansing, Michigan, USA
    PLoS ONE 4:e7874. 2009
    ....
  17. ncbi request reprint Differential phenotypes of active site and human autosomal dominant progressive external ophthalmoplegia mutations in Drosophila mitochondrial DNA helicase expressed in Schneider cells
    Yuichi Matsushima
    Department of Biochemistry and Molecular Biology, Michigan State University, East Lansing, Michigan 48824, USA
    J Biol Chem 282:9436-44. 2007
    ..Our dominant negative analysis of d-mtDNA helicase in cultured cells provides a tractable model for understanding human autosomal dominant progressive external ophthalmoplegia mutations...
  18. pmc Dynamic effects of cofactors and DNA on the oligomeric state of human mitochondrial DNA helicase
    Tawn D Ziebarth
    Department of Biochemistry and Molecular Biology and the Center for Mitochondrial Science and Medicine, Michigan State University, East Lansing, Michigan 49924 1319, USA
    J Biol Chem 285:14639-47. 2010
    ....
  19. pmc Physical analysis of recombinant forms of the human mitochondrial DNA helicase
    Magdalena M Makowska-Grzyska
    Department of Biochemistry and Molecular Biology and Center for Mitochondrial Science and Medicine, Michigan State University, East Lansing, MI 48823, USA
    Methods 51:411-5. 2010
    ..Despite their relatively common usage, we believe that their versatility makes these techniques particularly helpful in the characterization of oligomeric proteins...
  20. pmc The accessory subunit of DNA polymerase gamma is essential for mitochondrial DNA maintenance and development in Drosophila melanogaster
    Balaji Iyengar
    Department of Biology, McMaster University, Hamilton, ON, Canada L8S 4K1
    Proc Natl Acad Sci U S A 99:4483-8. 2002
    ..We discuss our results in the context of current hypotheses for the function of the accessory subunit in mtDNA replication...
  21. ncbi request reprint A novel processive mechanism for DNA synthesis revealed by structure, modeling and mutagenesis of the accessory subunit of human mitochondrial DNA polymerase
    Li Fan
    Life Sciences Division, Lawrence Berkeley National Laboratory, Berkeley, CA 94720, USA Department of Molecular Biology, The Scripps Research Institute, La Jolla, CA 92034, USA
    J Mol Biol 358:1229-43. 2006
    ....
  22. ncbi request reprint Functional defects due to spacer-region mutations of human mitochondrial DNA polymerase in a family with an ataxia-myopathy syndrome
    Petri T Luoma
    Programme of Neurosciences, Biomedicum Helsinki, Helsinki University, Finland
    Hum Mol Genet 14:1907-20. 2005
    ....
  23. doi request reprint Drosophila melanogaster as a model system to study mitochondrial biology
    Miguel Angel Fernández-Moreno
    Departamento de Bioquimica, Instituto de Investagaciones Biomédicas Alberto Sols CSIC UAM, Facultad de Medicina, Universidad Autonoma de Madrid, Spain
    Methods Mol Biol 372:33-49. 2007
    ....
  24. pmc Mitochondrial transcription factor B2 is essential for metabolic function in Drosophila melanogaster development
    Cristina Adán
    Departamento de Bioquimica, Instituto de Investigaciones Biomedicas Alberto Sols CSIC UAM, CIBERER ISCIII, Facultad de Medicina, Universidad Autonoma de Madrid, C Arzobispo Morcillo 4, E 28029 Madrid, Spain
    J Biol Chem 283:12333-42. 2008
    ....
  25. ncbi request reprint Modeling human mitochondrial diseases in flies
    Alvaro Sánchez-Martínez
    Departamento de Bioquimica, Instituto de Investigaciones Biomédicas Alberto Sols CSIC UAM Facultad de Medicina, Universidad Autonoma de Madrid, Arzobispo Morcillo 4, E 28029 Madrid, Spain
    Biochim Biophys Acta 1757:1190-8. 2006
    ..We also describe several genetic approaches for gene manipulation in flies, including the recently developed method of targeted mutagenesis by recombinational knock-in...
  26. ncbi request reprint A Drosophila model of mitochondrial DNA replication: proteins, genes and regulation
    Rafael Garesse
    Departamento de Bioquimica, Instituto de Investigaciones Biomedicas Alberto Sols CSIC UAM, Facultad de Medicina, Universidad Autonoma de Madrid, Madrid, Spain
    IUBMB Life 57:555-61. 2005
    ..It also presents a survey of cell and animal models under development to mimic the pathophysiology of human mitochondrial disorders...
  27. ncbi request reprint Over-expression of the catalytic core of mitochondrial DNA (mtDNA) polymerase in the nervous system of Drosophila melanogaster reduces median life span by inducing mtDNA depletion
    Francisco Martinez-Azorin
    Departamento de Bioquimica, Instituto de Investigaciones Biomedicas Alberto Sols CSIC UAM, Facultad de Medicina CIBERER ISCIII, Universidad Autonoma de Madrid, Madrid, Spain
    J Neurochem 105:165-76. 2008
    ..Our data establish an animal model that mimics some of the features of human mtDNA depletion syndromes...

Research Grants17

  1. Mitochondrial DNA Replisome: Mechanism, Structure and Physiology
    LAURIE SIMON KAGUNI; Fiscal Year: 2010
    ..Therefore, a clear understanding of mitochondrial biogenesis and energy production and how these processes are deregulated in common disease states, is essential to treating them effectively (and economically). ..
  2. MITOCHONDRIAL DNA POLYMERASE: MECHANISM AND STRUCTURE
    LAURIE KAGUNI; Fiscal Year: 2007
    ....
  3. MITOCHONDRIAL DNA POLYMERASE: MECHANISM AND STRUCTURE
    LAURIE KAGUNI; Fiscal Year: 2006
    ....
  4. MITOCHONDRIAL DNA POLYMERASE: MECHANISM AND STRUCTURE
    LAURIE KAGUNI; Fiscal Year: 2005
    ....
  5. MITOCHONDRIAL DNA POLYMERASE: MECHANISM AND STRUCTURE
    LAURIE KAGUNI; Fiscal Year: 2004
    ....
  6. MITOCHONDRIAL DNA POLYMERASE: MECHANISM AND STRUCTURE
    LAURIE KAGUNI; Fiscal Year: 2003
    ....
  7. MITOCHONDRIAL DNA POLYMERASE: MECHANISM AND STRUCTURE
    LAURIE KAGUNI; Fiscal Year: 2002
    ....
  8. MITOCHONDRIAL DNA POLYMERASE: MECHANISM AND STRUCTURE
    LAURIE KAGUNI; Fiscal Year: 2001
    ....
  9. MITOCHONDRIAL DNA POLYMERASE--MECHANISM AND STRUCTURE
    LAURIE KAGUNI; Fiscal Year: 2000
    ..To initiate the development of a transgenic fly model to examine the relationship between Pol gamma function, mitochondrial DNA replication fidelity, aging and disease. ..
  10. MITOCHONDRIAL DNA POLYMERASE--MECHANISM AND STRUCTURE
    LAURIE KAGUNI; Fiscal Year: 1993
    ..A detailed analysis of the key enzyme involved in mitochondrial DNA replication, gamma polymerase, will represent a major contribution toward an eventual understanding of mitochondrial biogenesis...
  11. MITOCHONDRIAL DNA POLYMERASE--MECHANISM AND STRUCTURE
    LAURIE KAGUNI; Fiscal Year: 1992
    ..A detailed analysis of the key enzyme involved in mitochondrial DNA replication, gamma polymerase, will represent a major contribution toward an eventual understanding of mitochondrial biogenesis...
  12. MITOCHONDRIAL DNA POLYMERASE--MECHANISM AND STRUCTURE
    LAURIE KAGUNI; Fiscal Year: 1999
    ..To initiate the development of a transgenic fly model to examine the relationship between Pol gamma function, mitochondrial DNA replication fidelity, aging and disease. ..
  13. MITOCHONDRIAL DNA POLYMERASE: MECHANISM AND STRUCTURE
    LAURIE KAGUNI; Fiscal Year: 2009
    ....