PubRank

Leonard Petrucelli

Author PubWeight™ 113.78‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS. Neuron 2013 5.91
2 Inhibitors of leucine-rich repeat kinase-2 protect against models of Parkinson's disease. Nat Med 2010 4.45
3 Novel mutations in TARDBP (TDP-43) in patients with familial amyotrophic lateral sclerosis. PLoS Genet 2008 3.66
4 Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity. Proc Natl Acad Sci U S A 2009 3.65
5 Progranulin mediates caspase-dependent cleavage of TAR DNA binding protein-43. J Neurosci 2007 3.52
6 The high-affinity HSP90-CHIP complex recognizes and selectively degrades phosphorylated tau client proteins. J Clin Invest 2007 3.35
7 RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention. Neuron 2013 3.25
8 Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion. Sci Transl Med 2013 2.89
9 Structural determinants of the cellular localization and shuttling of TDP-43. J Cell Sci 2008 2.79
10 Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice. J Neurosci 2010 2.60
11 Tar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue. PLoS One 2010 2.49
12 Association between repeat sizes and clinical and pathological characteristics in carriers of C9ORF72 repeat expansions (Xpansize-72): a cross-sectional cohort study. Lancet Neurol 2013 2.29
13 Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons. Acta Neuropathol 2013 2.26
14 Accelerated lipofuscinosis and ubiquitination in granulin knockout mice suggest a role for progranulin in successful aging. Am J Pathol 2010 2.20
15 Deletion of the ubiquitin ligase CHIP leads to the accumulation, but not the aggregation, of both endogenous phospho- and caspase-3-cleaved tau species. J Neurosci 2006 1.98
16 Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS. Acta Neuropathol 2013 1.95
17 TMEM106B protects C9ORF72 expansion carriers against frontotemporal dementia. Acta Neuropathol 2014 1.90
18 HSP induction mediates selective clearance of tau phosphorylated at proline-directed Ser/Thr sites but not KXGS (MARK) sites. FASEB J 2006 1.75
19 C9ORF72 repeat expansions in cases with previously identified pathogenic mutations. Neurology 2013 1.70
20 Akt and CHIP coregulate tau degradation through coordinated interactions. Proc Natl Acad Sci U S A 2008 1.66
21 CHIP regulates leucine-rich repeat kinase-2 ubiquitination, degradation, and toxicity. Proc Natl Acad Sci U S A 2009 1.64
22 Neurotoxic effects of TDP-43 overexpression in C. elegans. Hum Mol Genet 2010 1.56
23 The role of tau in neurodegeneration. Mol Neurodegener 2009 1.54
24 Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress. Acta Neuropathol 2014 1.54
25 Reduced C9orf72 gene expression in c9FTD/ALS is caused by histone trimethylation, an epigenetic event detectable in blood. Acta Neuropathol 2013 1.52
26 TDP-43 is a key player in the clinical features associated with Alzheimer's disease. Acta Neuropathol 2014 1.47
27 Identification of potential protein interactors of Lrrk2. Parkinsonism Relat Disord 2007 1.44
28 Homozygosity for the C9orf72 GGGGCC repeat expansion in frontotemporal dementia. Acta Neuropathol 2013 1.41
29 Molecular pathogenesis of Parkinson disease. Arch Neurol 2005 1.36
30 Staging TDP-43 pathology in Alzheimer's disease. Acta Neuropathol 2013 1.36
31 Mechanisms of toxicity in C9FTLD/ALS. Acta Neuropathol 2014 1.35
32 Caught in the act: alpha-synuclein is the culprit in Parkinson's disease. Neuron 2003 1.33
33 Development of a high throughput drug screening assay for the detection of changes in tau levels -- proof of concept with HSP90 inhibitors. Curr Alzheimer Res 2005 1.33
34 Dipeptide repeat proteins are present in the p62 positive inclusions in patients with frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72. Acta Neuropathol Commun 2013 1.32
35 Brain CHIP: removing the culprits in neurodegenerative disease. Trends Mol Med 2006 1.27
36 Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice. Mol Neurodegener 2011 1.20
37 Targeting Abeta and tau in Alzheimer's disease, an early interim report. Exp Neurol 2009 1.18
38 Progranulin regulates neuronal outgrowth independent of sortilin. Mol Neurodegener 2012 1.14
39 The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons. Hum Mol Genet 2013 1.11
40 Proteasome inhibition and Parkinson's disease modeling. Ann Neurol 2006 1.10
41 Disruption of protein quality control in Parkinson's disease. Cold Spring Harb Perspect Med 2012 1.07
42 Misregulation of human sortilin splicing leads to the generation of a nonfunctional progranulin receptor. Proc Natl Acad Sci U S A 2012 1.07
43 RNA-mediated toxicity in neurodegenerative disease. Mol Cell Neurosci 2012 1.07
44 Three repeat isoforms of tau inhibit assembly of four repeat tau filaments. PLoS One 2010 1.06
45 Phosphorylation regulates proteasomal-mediated degradation and solubility of TAR DNA binding protein-43 C-terminal fragments. Mol Neurodegener 2010 1.06
46 Acetylation of the KXGS motifs in tau is a critical determinant in modulation of tau aggregation and clearance. Hum Mol Genet 2013 1.05
47 Gene dosage and pathogenesis of Parkinson's disease. Trends Mol Med 2005 1.04
48 A critical evaluation of the ubiquitin-proteasome system in Parkinson's disease. Biochim Biophys Acta 2009 1.02
49 The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation. Hum Mol Genet 2013 1.02
50 Loss of HDAC6, a novel CHIP substrate, alleviates abnormal tau accumulation. Hum Mol Genet 2012 1.00
51 ALS-linked mutations enlarge TDP-43-enriched neuronal RNA granules in the dendritic arbor. J Neurosci 2014 1.00
52 Novel mutation in MAPT exon 13 (p.N410H) causes corticobasal degeneration. Acta Neuropathol 2014 0.98
53 Neuronal sensitivity to TDP-43 overexpression is dependent on timing of induction. Acta Neuropathol 2012 0.97
54 TDP-1/TDP-43 regulates stress signaling and age-dependent proteotoxicity in Caenorhabditis elegans. PLoS Genet 2012 0.97
55 The ubiquitin-proteasome reporter GFPu does not accumulate in neurons of the R6/2 transgenic mouse model of Huntington's disease. PLoS One 2009 0.97
56 Current strategies for the treatment of Alzheimer's disease and other tauopathies. Expert Opin Ther Targets 2006 0.95
57 TDP-43 in neurodegenerative disorders. Expert Opin Biol Ther 2008 0.94
58 Targeted manipulation of the sortilin-progranulin axis rescues progranulin haploinsufficiency. Hum Mol Genet 2013 0.93
59 Selective forelimb impairment in rats expressing a pathological TDP-43 25 kDa C-terminal fragment to mimic amyotrophic lateral sclerosis. Mol Ther 2013 0.92
60 Pharmacologic reductions of total tau levels; implications for the role of microtubule dynamics in regulating tau expression. Mol Neurodegener 2006 0.92
61 Progranulin: an emerging target for FTLD therapies. Brain Res 2012 0.90
62 Progressive amnestic dementia, hippocampal sclerosis, and mutation in C9ORF72. Acta Neuropathol 2013 0.89
63 Aging is not associated with proteasome impairment in UPS reporter mice. PLoS One 2009 0.88
64 Rodent models of TDP-43 proteinopathy: investigating the mechanisms of TDP-43-mediated neurodegeneration. J Mol Neurosci 2011 0.87
65 TARDBP mutations in Parkinson's disease. Parkinsonism Relat Disord 2012 0.85
66 c9RAN translation: a potential therapeutic target for the treatment of amyotrophic lateral sclerosis and frontotemporal dementia. Expert Opin Ther Targets 2013 0.85
67 TARDBP mutation analysis in TDP-43 proteinopathies and deciphering the toxicity of mutant TDP-43. J Alzheimers Dis 2013 0.85
68 Genetic modifiers in carriers of repeat expansions in the C9ORF72 gene. Mol Neurodegener 2014 0.84
69 Parkin is protective for substantia nigra dopamine neurons in a tau gene transfer neurodegeneration model. Neurosci Lett 2006 0.84
70 A novel tau mutation, p.K317N, causes globular glial tauopathy. Acta Neuropathol 2015 0.84
71 Robust cytoplasmic accumulation of phosphorylated TDP-43 in transgenic models of tauopathy. Acta Neuropathol 2013 0.83
72 Tau triage decisions mediated by the chaperone network. J Alzheimers Dis 2013 0.76
73 Progranulin axis and recent developments in frontotemporal lobar degeneration. Alzheimers Res Ther 2012 0.76
74 "What kills neurons in neurodegenerative diseases?", a review series in an open access journal. Mol Neurodegener 2009 0.75
75 Commentary on "Cytoskeletal modulators and pleiotropic strategies for Alzheimer drug discovery." The last stand: the dichotomy of chaperone function in Alzheimer's disease. Alzheimers Dement 2006 0.75
76 Does obesity-induced τ phosphorylation tip the scale toward dementia? Diabetes 2013 0.75
77 Discovery of a Biomarker and Lead Small Molecules to Target r(GGGGCC)-Associated Defects in c9FTD/ALS. Neuron 2014 0.75