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tampereen yliopisto: lääketieteen ja biotieteiden tiedekunta: tutkimus: laurie kaguni - terveiden ja sairaiden mitokondrioiden biogeneesi:
Lääketieteen ja biotieteiden tiedekunta FinMIT Centre of Exellence in research on  mitochondrial disease and ageing University of Tampere Institute of Biomedical Technology - IBT
Laurie Kaguni - Terveiden ja sairaiden mitokondrioiden biogeneesi

Aiempia julkaisuja

Ciesielski GL, Bermek O, Rosado-Ruiz FA, Hovde SL, Neitzke OJ, Griffith JD, Kaguni LS
Mitochondrial Single-stranded DNA-binding Proteins Stimulate the Activity of DNA Polymerase γ by Organization of the Template DNA.
J Biol Chem 2015 ;290(48)28697-707
PMID

Oliveira MT, Haukka J, Kaguni LS
Evolution of the metazoan mitochondrial replicase.
Genome Biol Evol 2015 ;7(4)943-59
PMID

Zabalza R, Nurminen A, Kaguni LS, Garesse R, Gallardo ME, Bornstein B
Co-occurrence of four nucleotide changes associated with an adult mitochondrial ataxia phenotype.
BMC Res Notes 2014 ;7883
PMID

Baqri RM, Pietron AV, Gokhale RH, Turner BA, Kaguni LS, Shingleton AW, Kunes S, Miller KE
Mitochondrial chaperone TRAP1 activates the mitochondrial UPR and extends healthspan in Drosophila.
Mech Ageing Dev 2014 ;141-142C35-45
PMID

Stiban J, Farnum GA, Hovde SL, Kaguni LS
The N-terminal domain of the Drosophila mitochondrial replicative DNA helicase contains an iron-sulfur cluster and binds DNA.
J Biol Chem 2014 ;289(35)24032-42
PMID

Farnum GA, Nurminen A, Kaguni LS
Mapping 136 pathogenic mutations into functional modules in human DNA polymerase γ establishes predictive genotype-phenotype correlations for the complete spectrum of POLG syndromes.
Biochim Biophys Acta 2014 ;1837(7)1113-21
PMID

Fernández-Moreno MA, Hernández R, Adán C, Roberti M, Bruni F, Polosa PL, Cantatore P, Matsushima Y, Kaguni LS, Garesse R
Drosophila nuclear factor DREF regulates the expression of the mitochondrial DNA helicase and mitochondrial transcription factor B2 but not the mitochondrial translation factor B1.
Biochim Biophys Acta 2013 ;1829(10)1136-46
PMID

Martínez-Azorín F, Calleja M, Hernández-Sierra R, Farr CL, Kaguni LS, Garesse R
Muscle-specific overexpression of the catalytic subunit of DNA polymerase γ induces pupal lethality in Drosophila melanogaster.
Arch Insect Biochem Physiol 2013 ;83(3)127-37
PMID

Sanchez-Martinez A, Calleja M, Peralta S, Matsushima Y, Hernandez-Sierra R, Whitworth AJ, Kaguni LS, Garesse R
Modeling pathogenic mutations of human twinkle in Drosophila suggests an apoptosis role in response to mitochondrial defects.
PLoS One 2012 ;7(8)e43954
PMID

Chen S, Oliveira MT, Sanz A, Kemppainen E, Fukuoh A, Schlicht B, Kaguni LS, Jacobs HT
A cytoplasmic suppressor of a nuclear mutation affecting mitochondrial functions in Drosophila.
Genetics 2012 ;192(2)483-93
PMID

Peralta S, Clemente P, Sánchez-Martínez A, Calleja M, Hernández-Sierra R, Matsushima Y, Adán C, Ugalde C, Fernández-Moreno MÁ, Kaguni LS, Garesse R
Coiled coil domain-containing protein 56 (CCDC56) is a novel mitochondrial protein essential for cytochrome c oxidase function.
J Biol Chem 2012 ;287(29)24174-85
PMID

Oliveira MT, Kaguni LS
Reduced stimulation of recombinant DNA polymerase γ and mitochondrial DNA (mtDNA) helicase by variants of mitochondrial single-stranded DNA-binding protein (mtSSB) correlates with defects in mtDNA replication in animal cells.
J Biol Chem 2011 ;286(47)40649-58
PMID

Euro L, Farnum GA, Palin E, Suomalainen A, Kaguni LS
Clustering of Alpers disease mutations and catalytic defects in biochemical variants reveal new features of molecular mechanism of the human mitochondrial replicase, Pol γ.
Nucleic Acids Res 2011 ;39(21)9072-84
PMID

Oliveira MT, Kaguni LS
Functional roles of the N- and C-terminal regions of the human mitochondrial single-stranded DNA-binding protein.
PLoS One 2010 ;5(10)e15379
PMID

Matsushima Y, Goto Y, Kaguni LS
Mitochondrial Lon protease regulates mitochondrial DNA copy number and transcription by selective degradation of mitochondrial transcription factor A (TFAM).
Proc Natl Acad Sci U S A 2010 ;107(43)18410-5
PMID

Makowska-Grzyska MM, Ziebarth TD, Kaguni LS
Physical analysis of recombinant forms of the human mitochondrial DNA helicase.
Methods 2010 ;51(4)411-5
PMID

Ziebarth TD, Gonzalez-Soltero R, Makowska-Grzyska MM, Núñez-Ramírez R, Carazo JM, Kaguni LS
Dynamic effects of cofactors and DNA on the oligomeric state of human mitochondrial DNA helicase.
J Biol Chem 2010 ;285(19)14639-47
PMID

Dallmann HG, Fackelmayer OJ, Tomer G, Chen J, Wiktor-Becker A, Ferrara T, Pope C, Oliveira MT, Burgers PM, Kaguni LS, McHenry CS
Parallel multiplicative target screening against divergent bacterial replicases: identification of specific inhibitors with broad spectrum potential.
Biochemistry 2010 ;49(11)2551-62
PMID

Palin EJ, Lesonen A, Farr CL, Euro L, Suomalainen A, Kaguni LS
Functional analysis of H. sapiens DNA polymerase gamma spacer mutation W748S with and without common variant E1143G.
Biochim Biophys Acta 2010 ;1802(6)545-51
PMID

Oliveira MT, Garesse R, Kaguni LS
Animal models of mitochondrial DNA transactions in disease and ageing.
Exp Gerontol 2010 ;45(7-8)489-502
PMID

Baqri RM, Turner BA, Rheuben MB, Hammond BD, Kaguni LS, Miller KE
Disruption of mitochondrial DNA replication in Drosophila increases mitochondrial fast axonal transport in vivo.
PLoS One 2009 ;4(11)e7874
PMID

Weinreb V, Li L, Campbell CL, Kaguni LS, Carter CW
Mg2+-assisted catalysis by B. stearothermophilus TrpRS is promoted by allosteric effects.
Structure 2009 ;17(7)952-64
PMID

Ziebarth TD, Kaguni LS
Purification strategy for recombinant forms of the human mitochondrial DNA helicase.
Methods Mol Biol 2009 ;554121-6
PMID

Oliveira MT, Kaguni LS
Comparative purification strategies for Drosophila and human mitochondrial DNA replication proteins: DNA polymerase gamma and mitochondrial single-stranded DNA-binding protein.
Methods Mol Biol 2009 ;55437-58
PMID

Matsushima Y, Kaguni LS
Functional importance of the conserved N-terminal domain of the mitochondrial replicative DNA helicase.
Biochim Biophys Acta 2009 ;1787(5)290-5
PMID

Matsushima Y, Farr CL, Fan L, Kaguni LS
Physiological and biochemical defects in carboxyl-terminal mutants of mitochondrial DNA helicase.
J Biol Chem 2008 ;283(35)23964-71
PMID

Adán C, Matsushima Y, Hernández-Sierra R, Marco-Ferreres R, Fernández-Moreno MA, González-Vioque E, Calleja M, Aragón JJ, Kaguni LS, Garesse R
Mitochondrial transcription factor B2 is essential for metabolic function in Drosophila melanogaster development.
J Biol Chem 2008 ;283(18)12333-42
PMID

Martínez-Azorín F, Calleja M, Hernández-Sierra R, Farr CL, Kaguni LS, Garesse R
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.
J Neurochem 2008 ;105(1)165-76
PMID

Matsushima Y, Adán C, Garesse R, Kaguni LS
Functional analysis by inducible RNA interference in Drosophila melanogaster.
Methods Mol Biol 2007 ;372207-17
PMID

Fernández-Moreno MA, Farr CL, Kaguni LS, Garesse R
Drosophila melanogaster as a model system to study mitochondrial biology.
Methods Mol Biol 2007 ;37233-49
PMID

Ziebarth TD, Farr CL, Kaguni LS
Modular architecture of the hexameric human mitochondrial DNA helicase.
J Mol Biol 2007 ;367(5)1382-91
PMID

Matsushima Y, Kaguni LS
Differential phenotypes of active site and human autosomal dominant progressive external ophthalmoplegia mutations in Drosophila mitochondrial DNA helicase expressed in Schneider cells.
J Biol Chem 2007 ;282(13)9436-44
PMID

Fan L, Kim S, Farr CL, Schaefer KT, Randolph KM, Tainer JA, Kaguni LS
A novel processive mechanism for DNA synthesis revealed by structure, modeling and mutagenesis of the accessory subunit of human mitochondrial DNA polymerase.
J Mol Biol 2006 ;358(5)1229-43
PMID

Garesse R, Kaguni LS
A Drosophila model of mitochondrial DNA replication: proteins, genes and regulation.
IUBMB Life 2005 ;57(8)555-61
PMID

Luoma PT, Luo N, Löscher WN, Farr CL, Horvath R, Wanschitz J, Kiechl S, Kaguni LS, Suomalainen A
Functional defects due to spacer-region mutations of human mitochondrial DNA polymerase in a family with an ataxia-myopathy syndrome.
Hum Mol Genet 2005 ;14(14)1907-20
PMID

Matsushima Y, Adán C, Garesse R, Kaguni LS
Drosophila mitochondrial transcription factor B1 modulates mitochondrial translation but not transcription or DNA copy number in Schneider cells.
J Biol Chem 2005 ;280(17)16815-20
PMID

Luo N, Kaguni LS
Mutations in the spacer region of Drosophila mitochondrial DNA polymerase affect DNA binding, processivity, and the balance between Pol and Exo function.
J Biol Chem 2005 ;280(4)2491-7
PMID

Kaguni LS
DNA polymerase gamma, the mitochondrial replicase.
Annu Rev Biochem 2004 ;73293-320
PMID

Matsushima Y, Garesse R, Kaguni LS
Drosophila mitochondrial transcription factor B2 regulates mitochondrial DNA copy number and transcription in schneider cells.
J Biol Chem 2004 ;279(26)26900-5
PMID

Farr CL, Matsushima Y, Lagina AT, Luo N, Kaguni LS
Physiological and biochemical defects in functional interactions of mitochondrial DNA polymerase and DNA-binding mutants of single-stranded DNA-binding protein.
J Biol Chem 2004 ;279(17)17047-53
PMID

Farr CL, Kaguni LS
Purification strategies for Drosophila mtDNA replication proteins in native and recombinant form. Mitochondrial single-stranded DNA-binding protein.
Methods Mol Biol 2002 ;197285-94
PMID

Farr CL, Kaguni LS
Purification strategies for Drosophila mtDNA replication proteins in native and recombinant form. DNA polymerase gamma.
Methods Mol Biol 2002 ;197273-83
PMID

Iyengar B, Luo N, Farr CL, Kaguni LS, Campos AR
The accessory subunit of DNA polymerase gamma is essential for mitochondrial DNA maintenance and development in Drosophila melanogaster.
Proc Natl Acad Sci U S A 2002 ;99(7)4483-8
PMID

Ugalde C, Ochoa P, Pérez ML, Fernández-Moreno MA, Calleja M, Alahari A, Kaguni LS, Garesse R
Identification of a proximal promoter region critical for the expression of the beta-F1-ATPase gene during Drosophila melanogaster development.
Mitochondrion 2001 ;1(3)225-36
PMID

Maier D, Farr CL, Poeck B, Alahari A, Vogel M, Fischer S, Kaguni LS, Schneuwly S
Mitochondrial single-stranded DNA-binding protein is required for mitochondrial DNA replication and development in Drosophila melanogaster.
Mol Biol Cell 2001 ;12(4)821-30
PMID

Fan L, Kaguni LS
Multiple regions of subunit interaction in Drosophila mitochondrial DNA polymerase: three functional domains in the accessory subunit.
Biochemistry 2001 ;40(15)4780-91
PMID

Lefai E, Calleja M, Ruiz de Mena I, Lagina AT, Kaguni LS, Garesse R
Overexpression of the catalytic subunit of DNA polymerase gamma results in depletion of mitochondrial DNA in Drosophila melanogaster.
Mol Gen Genet 2000 ;264(1-2)37-46
PMID

Lefai E, Fernandez-Moreno MA, Alahari A, Kaguni LS, Garesse R
Differential regulation of the catalytic and accessory subunit genes of Drosophila mitochondrial DNA polymerase.
J Biol Chem 2000 ;275(42)33123-33
PMID

Lefai E, Fernández-Moreno MA, Kaguni LS, Garesse R
The highly compact structure of the mitochondrial DNA polymerase genomic region of Drosophila melanogaster: functional and evolutionary implications.
Insect Mol Biol 2000 ;9(3)315-22
PMID

Ruiz De Mena I, Lefai E, Garesse R, Kaguni LS
Regulation of mitochondrial single-stranded DNA-binding protein gene expression links nuclear and mitochondrial DNA replication in drosophila.
J Biol Chem 2000 ;275(18)13628-36
PMID

 
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Tampereen yliopisto

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