PubRank

Koji Yamanaka

Author PubWeight™ 38.44‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Onset and progression in inherited ALS determined by motor neurons and microglia. Science 2006 9.42
2 Antisense oligonucleotide therapy for neurodegenerative disease. J Clin Invest 2006 3.00
3 Gene transfer demonstrates that muscle is not a primary target for non-cell-autonomous toxicity in familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A 2006 1.97
4 Complete loss of post-translational modifications triggers fibrillar aggregation of SOD1 in the familial form of amyotrophic lateral sclerosis. J Biol Chem 2008 1.69
5 Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS. Neuron 2010 1.65
6 A seeding reaction recapitulates intracellular formation of Sarkosyl-insoluble transactivation response element (TAR) DNA-binding protein-43 inclusions. J Biol Chem 2011 1.48
7 Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death. Proc Natl Acad Sci U S A 2008 1.45
8 Virus-delivered small RNA silencing sustains strength in amyotrophic lateral sclerosis. Ann Neurol 2005 1.44
9 Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice. Proc Natl Acad Sci U S A 2009 1.33
10 Spliceosome integrity is defective in the motor neuron diseases ALS and SMA. EMBO Mol Med 2013 1.22
11 Mutation-dependent polymorphism of Cu,Zn-superoxide dismutase aggregates in the familial form of amyotrophic lateral sclerosis. J Biol Chem 2010 1.15
12 Glial cells in amyotrophic lateral sclerosis. Neurol Res Int 2011 1.08
13 Blockade of gap junction hemichannel suppresses disease progression in mouse models of amyotrophic lateral sclerosis and Alzheimer's disease. PLoS One 2011 1.08
14 Loss of TDP-43 causes age-dependent progressive motor neuron degeneration. Brain 2013 1.05
15 Gigaxonin controls vimentin organization through a tubulin chaperone-independent pathway. Hum Mol Genet 2009 1.04
16 Accelerated disease onset with stabilized familial amyotrophic lateral sclerosis (ALS)-linked mutant TDP-43 proteins. J Biol Chem 2012 1.02
17 Disulfide scrambling describes the oligomer formation of superoxide dismutase (SOD1) proteins in the familial form of amyotrophic lateral sclerosis. J Biol Chem 2012 0.94
18 Intranuclear aggregation of mutant FUS/TLS as a molecular pathomechanism of amyotrophic lateral sclerosis. J Biol Chem 2013 0.92
19 Novel missense mutation in ALS2 gene results in infantile ascending hereditary spastic paralysis. Ann Neurol 2006 0.91
20 Heat-shock protein 105 interacts with and suppresses aggregation of mutant Cu/Zn superoxide dismutase: clues to a possible strategy for treating ALS. J Neurochem 2007 0.90
21 Disease causing mutants of TDP-43 nucleic acid binding domains are resistant to aggregation and have increased stability and half-life. Proc Natl Acad Sci U S A 2014 0.89
22 Intracellular seeded aggregation of mutant Cu,Zn-superoxide dismutase associated with amyotrophic lateral sclerosis. FEBS Lett 2013 0.87
23 E6-AP association promotes SOD1 aggresomes degradation and suppresses toxicity. Neurobiol Aging 2012 0.86
24 SIRT1 overexpression ameliorates a mouse model of SOD1-linked amyotrophic lateral sclerosis via HSF1/HSP70i chaperone system. Mol Brain 2014 0.82
25 Determinants of rapid disease progression in ALS. Neurology 2005 0.75
26 [Neuroinflammation in amyotrophic lateral sclerosis]. Rinsho Shinkeigaku 2014 0.75
27 [Paraneoplastic motor neuron disease]. Nihon Rinsho 2015 0.75