PubRank

Robert J Ferrante

Author PubWeight™ 104.10‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 A call for transparent reporting to optimize the predictive value of preclinical research. Nature 2012 14.63
2 Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in mice. Nature 2002 4.21
3 Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage. Hum Mol Genet 2007 2.54
4 Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease. J Biol Chem 2004 2.43
5 Histone deacetylase inhibitors prevent oxidative neuronal death independent of expanded polyglutamine repeats via an Sp1-dependent pathway. Proc Natl Acad Sci U S A 2003 2.21
6 Minocycline inhibits caspase-independent and -dependent mitochondrial cell death pathways in models of Huntington's disease. Proc Natl Acad Sci U S A 2003 2.05
7 Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice. J Comp Neurol 2005 1.95
8 Translational control of inducible nitric oxide synthase expression by arginine can explain the arginine paradox. Proc Natl Acad Sci U S A 2003 1.92
9 Therapeutic effects of cystamine in a murine model of Huntington's disease. J Neurosci 2002 1.92
10 Sodium phenylbutyrate prolongs survival and regulates expression of anti-apoptotic genes in transgenic amyotrophic lateral sclerosis mice. J Neurochem 2005 1.91
11 Transcriptional therapy with the histone deacetylase inhibitor trichostatin A ameliorates experimental autoimmune encephalomyelitis. J Neuroimmunol 2005 1.89
12 ESET/SETDB1 gene expression and histone H3 (K9) trimethylation in Huntington's disease. Proc Natl Acad Sci U S A 2006 1.82
13 Ciliogenesis is regulated by a huntingtin-HAP1-PCM1 pathway and is altered in Huntington disease. J Clin Invest 2011 1.79
14 Transcriptional modulator H2A histone family, member Y (H2AFY) marks Huntington disease activity in man and mouse. Proc Natl Acad Sci U S A 2011 1.76
15 Sp1 and Sp3 are oxidative stress-inducible, antideath transcription factors in cortical neurons. J Neurosci 2003 1.72
16 Mitochondrial loss, dysfunction and altered dynamics in Huntington's disease. Hum Mol Genet 2010 1.62
17 Experimental therapeutics in transgenic mouse models of Huntington's disease. Nat Rev Neurosci 2004 1.58
18 Antioxidants modulate mitochondrial PKA and increase CREB binding to D-loop DNA of the mitochondrial genome in neurons. Proc Natl Acad Sci U S A 2005 1.42
19 In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects. Hum Mol Genet 2010 1.34
20 Cystamine increases L-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation. J Neurochem 2004 1.33
21 Mice lacking alpha-synuclein are resistant to mitochondrial toxins. Neurobiol Dis 2005 1.30
22 Mitochondrial cyclic AMP response element-binding protein (CREB) mediates mitochondrial gene expression and neuronal survival. J Biol Chem 2005 1.25
23 Combination therapy with coenzyme Q10 and creatine produces additive neuroprotective effects in models of Parkinson's and Huntington's diseases. J Neurochem 2009 1.22
24 Pyrimidine-2,4,6-trione derivatives and their inhibition of mutant SOD1-dependent protein aggregation. Toward a treatment for amyotrophic lateral sclerosis. J Med Chem 2011 1.22
25 Sp1 is up-regulated in cellular and transgenic models of Huntington disease, and its reduction is neuroprotective. J Biol Chem 2006 1.21
26 Dose ranging and efficacy study of high-dose coenzyme Q10 formulations in Huntington's disease mice. Biochim Biophys Acta 2006 1.21
27 Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice. J Neurochem 2003 1.20
28 The sirtuin 2 inhibitor AK-7 is neuroprotective in Huntington's disease mouse models. Cell Rep 2012 1.19
29 Inhibitors of cytochrome c release with therapeutic potential for Huntington's disease. J Neurosci 2008 1.17
30 ADME-guided design and synthesis of aryloxanyl pyrazolone derivatives to block mutant superoxide dismutase 1 (SOD1) cytotoxicity and protein aggregation: potential application for the treatment of amyotrophic lateral sclerosis. J Med Chem 2011 1.16
31 Modulation of nucleosome dynamics in Huntington's disease. Hum Mol Genet 2007 1.13
32 Identification of compounds protective against G93A-SOD1 toxicity for the treatment of amyotrophic lateral sclerosis. Amyotroph Lateral Scler 2010 1.12
33 Depletion of wild-type huntingtin in mouse models of neurologic diseases. J Neurochem 2003 1.12
34 Evidence of oxidant damage in Huntington's disease: translational strategies using antioxidants. Ann N Y Acad Sci 2008 1.12
35 Conformation-sensitive antibodies against alzheimer amyloid-beta by immunization with a thioredoxin-constrained B-cell epitope peptide. J Biol Chem 2007 1.08
36 Combination therapy using minocycline and coenzyme Q10 in R6/2 transgenic Huntington's disease mice. Biochim Biophys Acta 2005 1.08
37 Huntington's disease of the endocrine pancreas: insulin deficiency and diabetes mellitus due to impaired insulin gene expression. Neurobiol Dis 2002 1.07
38 Neuroprotective effects of synaptic modulation in Huntington's disease R6/2 mice. J Neurosci 2007 1.07
39 Cdc42-interacting protein 4 binds to huntingtin: neuropathologic and biological evidence for a role in Huntington's disease. Proc Natl Acad Sci U S A 2003 1.05
40 Phase 2 study of sodium phenylbutyrate in ALS. Amyotroph Lateral Scler 2009 1.05
41 Translating therapies for Huntington's disease from genetic animal models to clinical trials. NeuroRx 2004 1.05
42 Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington's disease. J Neurochem 2005 1.04
43 Therapeutic attenuation of mitochondrial dysfunction and oxidative stress in neurotoxin models of Parkinson's disease. Biochim Biophys Acta 2008 1.03
44 Cytochrome C and caspase-9 expression in Huntington's disease. Neuromolecular Med 2002 1.03
45 Arylsulfanyl pyrazolones block mutant SOD1-G93A aggregation. Potential application for the treatment of amyotrophic lateral sclerosis. Bioorg Med Chem 2010 0.99
46 The melatonin MT1 receptor axis modulates mutant Huntingtin-mediated toxicity. J Neurosci 2011 0.96
47 Safety and tolerability of high-dosage coenzyme Q10 in Huntington's disease and healthy subjects. Mov Disord 2010 0.96
48 Role of cyclooxygenase-2 induction by transcription factor Sp1 and Sp3 in neuronal oxidative and DNA damage response. FASEB J 2006 0.96
49 Combined riluzole and sodium phenylbutyrate therapy in transgenic amyotrophic lateral sclerosis mice. Amyotroph Lateral Scler 2009 0.96
50 Nortriptyline delays disease onset in models of chronic neurodegeneration. Eur J Neurosci 2007 0.96
51 Mitochondrial nuclear receptors and transcription factors: who's minding the cell? J Neurosci Res 2008 0.95
52 Sequential activation of individual caspases, and of alterations in Bcl-2 proapoptotic signals in a mouse model of Huntington's disease. J Neurochem 2003 0.95
53 Monoallele deletion of CBP leads to pericentromeric heterochromatin condensation through ESET expression and histone H3 (K9) methylation. Hum Mol Genet 2008 0.94
54 Prophylactic creatine administration mediates neuroprotection in cerebral ischemia in mice. J Neurosci 2004 0.94
55 Huntington's disease: progress and potential in the field. Expert Opin Investig Drugs 2007 0.92
56 Reduced creatine kinase as a central and peripheral biomarker in Huntington's disease. Biochim Biophys Acta 2010 0.91
57 Activation of Ets-2 by oxidative stress induces Bcl-xL expression and accounts for glial survival in amyotrophic lateral sclerosis. FASEB J 2009 0.90
58 SCAMP5 links endoplasmic reticulum stress to the accumulation of expanded polyglutamine protein aggregates via endocytosis inhibition. J Biol Chem 2009 0.90
59 MAP kinase phosphatase 1 (MKP-1/DUSP1) is neuroprotective in Huntington's disease via additive effects of JNK and p38 inhibition. J Neurosci 2013 0.90
60 Mice overexpressing 70-kDa heat shock protein show increased resistance to malonate and 3-nitropropionic acid. Exp Neurol 2002 0.89
61 Emerging chemotherapeutic strategies for Huntington's disease. Expert Opin Emerg Drugs 2005 0.88
62 Anti-inflammatory treatment with acetylsalicylate or rofecoxib is not neuroprotective in Huntington's disease transgenic mice. Neurobiol Dis 2004 0.88
63 Cyclohexane 1,3-diones and their inhibition of mutant SOD1-dependent protein aggregation and toxicity in PC12 cells. Bioorg Med Chem 2011 0.87
64 Increased survival and neuroprotective effects of BN82451 in a transgenic mouse model of Huntington's disease. J Neurochem 2003 0.86
65 Genetic and pharmacological inactivation of the adenosine A2A receptor attenuates 3-nitropropionic acid-induced striatal damage. J Neurochem 2004 0.86
66 Experimental models of HD and reflection on therapeutic strategies. Int Rev Neurobiol 2011 0.84
67 Melatonin inhibits the caspase-1/cytochrome c/caspase-3 cell death pathway, inhibits MT1 receptor loss and delays disease progression in a mouse model of amyotrophic lateral sclerosis. Neurobiol Dis 2013 0.83
68 Translational therapeutic strategies in amyotrophic lateral sclerosis. Mini Rev Med Chem 2007 0.83
69 The therapeutic role of creatine in Huntington's disease. Pharmacol Ther 2005 0.82
70 The neuroprotective role of creatine. Subcell Biochem 2007 0.81
71 Chiral cyclohexane 1,3-diones as inhibitors of mutant SOD1-dependent protein aggregation for the treatment of ALS. ACS Med Chem Lett 2012 0.81
72 A high-throughput screen to identify inhibitors of SOD1 transcription. Front Biosci (Elite Ed) 2012 0.80
73 Uridine ameliorates the pathological phenotype in transgenic G93A-ALS mice. Amyotroph Lateral Scler 2010 0.78
74 Mutant SOD1G93A in bone marrow-derived cells exacerbates 3-nitropropionic acid induced striatal damage in mice. Neurosci Lett 2007 0.77
75 Correction to "Chiral Cyclohexane 1,3-Diones as Inhibitors of Mutant SOD1-Dependent Protein Aggregation for the Treatment of ALS". ACS Med Chem Lett 2017 0.75