PubRank

Laurie S Kaguni

Author PubWeight™ 37.32‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Functional defects due to spacer-region mutations of human mitochondrial DNA polymerase in a family with an ataxia-myopathy syndrome. Hum Mol Genet 2005 1.58
2 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 1.38
3 Drosophila mitochondrial transcription factor B1 modulates mitochondrial translation but not transcription or DNA copy number in Schneider cells. J Biol Chem 2005 1.37
4 Drosophila mitochondrial transcription factor B2 regulates mitochondrial DNA copy number and transcription in schneider cells. J Biol Chem 2004 1.34
5 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 1.25
6 Modular architecture of the hexameric human mitochondrial DNA helicase. J Mol Biol 2007 1.21
7 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 1.11
8 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 1.09
9 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 1.08
10 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 1.05
11 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 2004 1.03
12 Functional roles of the N- and C-terminal regions of the human mitochondrial single-stranded DNA-binding protein. PLoS One 2010 1.00
13 Physiological and biochemical defects in carboxyl-terminal mutants of mitochondrial DNA helicase. J Biol Chem 2008 0.98
14 Mg2+-assisted catalysis by B. stearothermophilus TrpRS is promoted by allosteric effects. Structure 2009 0.96
15 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 0.94
16 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 2007 0.93
17 Dynamic effects of cofactors and DNA on the oligomeric state of human mitochondrial DNA helicase. J Biol Chem 2010 0.93
18 Animal models of mitochondrial DNA transactions in disease and ageing. Exp Gerontol 2010 0.93
19 Mitochondrial transcription factor B2 is essential for metabolic function in Drosophila melanogaster development. J Biol Chem 2008 0.91
20 Drosophila melanogaster as a model system to study mitochondrial biology. Methods Mol Biol 2007 0.90
21 Functional importance of the conserved N-terminal domain of the mitochondrial replicative DNA helicase. Biochim Biophys Acta 2008 0.89
22 A cytoplasmic suppressor of a nuclear mutation affecting mitochondrial functions in Drosophila. Genetics 2012 0.89
23 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 0.88
24 A Drosophila model of mitochondrial DNA replication: proteins, genes and regulation. IUBMB Life 2005 0.87
25 Matrix proteases in mitochondrial DNA function. Biochim Biophys Acta 2011 0.87
26 Disruption of mitochondrial DNA replication in Drosophila increases mitochondrial fast axonal transport in vivo. PLoS One 2009 0.87
27 Parallel multiplicative target screening against divergent bacterial replicases: identification of specific inhibitors with broad spectrum potential. Biochemistry 2010 0.86
28 Modeling pathogenic mutations of human twinkle in Drosophila suggests an apoptosis role in response to mitochondrial defects. PLoS One 2012 0.86
29 Modeling human mitochondrial diseases in flies. Biochim Biophys Acta 2006 0.85
30 Functional analysis of H. sapiens DNA polymerase gamma spacer mutation W748S with and without common variant E1143G. Biochim Biophys Acta 2010 0.81
31 Coiled coil domain-containing protein 56 (CCDC56) is a novel mitochondrial protein essential for cytochrome c oxidase function. J Biol Chem 2012 0.81
32 Purification strategy for recombinant forms of the human mitochondrial DNA helicase. Methods Mol Biol 2009 0.80
33 Purification strategies for Drosophila mtDNA replication proteins in native and recombinant form. Mitochondrial single-stranded DNA-binding protein. Methods Mol Biol 2002 0.79
34 Functional analysis by inducible RNA interference in Drosophila melanogaster. Methods Mol Biol 2007 0.78
35 Purification strategies for Drosophila mtDNA replication proteins in native and recombinant form. DNA polymerase gamma. Methods Mol Biol 2002 0.78
36 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 0.78
37 Physical analysis of recombinant forms of the human mitochondrial DNA helicase. Methods 2010 0.76
38 Muscle-specific overexpression of the catalytic subunit of DNA polymerase γ induces pupal lethality in Drosophila melanogaster. Arch Insect Biochem Physiol 2013 0.75
39 Co-occurrence of four nucleotide changes associated with an adult mitochondrial ataxia phenotype. BMC Res Notes 2014 0.75
40 Iron-Sulfur Clusters in Mitochondrial Metabolism: Multifaceted Roles of a Simple Cofactor. Biochemistry (Mosc) 2016 0.75