Research Topics
Genomes and Genes | R J FerranteSummaryAffiliation: Boston University Country: USA Publications
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Publications
Therapeutic effects of cystamine in a murine model of Huntington's diseaseAlpaslan Dedeoglu
Geriatric Research Education and Clinical Center, Bedford Veterans Affairs Medical Center, Bedford, Massachusetts 01730, USA
J Neurosci 22:8942-50. 2002..These findings are consistent with the hypothesis that transglutaminase activity may play a role in the pathogenesis of HD, and they identify cystamine as a potential therapeutic strategy for treating HD patients...- Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease miceRobert J Ferrante
Geriatric Research Education and Clinical Center, Bedford Veterans Affairs Medical Center, Bedford, Massachusetts 01730, USA
J Neurosci 23:9418-27. 2003.... 
Chemotherapy for the brain: the antitumor antibiotic mithramycin prolongs survival in a mouse model of Huntington's diseaseRobert J Ferrante
Geriatric Research and Education and Clinical Center, Veterans Administration Medical Center, Bedford, MA, USA
J Neurosci 24:10335-42. 2004..Because it is Food and Drug Administration-approved, mithramycin is a promising drug for the treatment of HD...- Dose ranging and efficacy study of high-dose coenzyme Q10 formulations in Huntington's disease miceKaren M Smith
Geriatric Research Education and Clinical Center, Bedford VA Medical Center, Bedford 01730, and Neurology Department, Boston University School of Medicine, MA 02180, USA
Biochim Biophys Acta 1762:616-26. 2006.... - Modulation of nucleosome dynamics in Huntington's diseaseEdward C Stack
Geriatric Research Education and Clinical Center, Bedford VA Medical Center, Bedford, MA 01730, USA
Hum Mol Genet 16:1164-75. 2007..These data show the ability of anthracycline compounds to rebalance epigenetic histone modification and, as such, may provide the rationale for the design of human clinical trials in HD patients... - Mouse models of Huntington's disease and methodological considerations for therapeutic trialsRobert J Ferrante
Geriatric Research Education and Clinical Center, Bedford Veterans Administration Medical Center, Bedford, Massachusetts 01730, USA
Biochim Biophys Acta 1792:506-20. 2009..As such, a guide to assessing studies in mouse models and salient issues related to translation from mice to humans are included... - Neuroprotective effects of creatine in a transgenic mouse model of Huntington's diseaseR J Ferrante
Geriatric Research Education and Clinical Center, Bedford Veteran s Administration Medical Center, Bedford, Massachusetts 01730, USA
J Neurosci 20:4389-97. 2000..These results support a role of metabolic dysfunction in a transgenic mouse model of HD and suggest a novel therapeutic strategy to slow the pathological process... - Therapeutic efficacy of EGb761 (Gingko biloba extract) in a transgenic mouse model of amyotrophic lateral sclerosisR J Ferrante
Geriatric Research Education and Clinical Center, Bedford VA Medical Center, MA 01730, USA
J Mol Neurosci 17:89-96. 2001..These findings provide evidence for a gender-specific neuroprotective effect of EGb761 in a transgenic model of ALS and suggest that EGb761 may be a potential effective treatment in patients with ALS... - Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's diseaseRobert J Ferrante
Geriatric Research Education and Clinical Center, Bedford Veterans Administration Medical Center, Bedford, Massachusetts 01730, USA
J Neurosci 22:1592-9. 2002.... - Sodium phenylbutyrate prolongs survival and regulates expression of anti-apoptotic genes in transgenic amyotrophic lateral sclerosis miceHoon Ryu
Geriatric Research Education and Clinical Center, Bedford VA Medical Center, Bedford, Massachusetts, USA
J Neurochem 93:1087-98. 2005..Phenylbutyrate may therefore provide a novel therapeutic approach for the treatment of patients with ALS... - Combination therapy using minocycline and coenzyme Q10 in R6/2 transgenic Huntington's disease miceEdward C Stack
Geriatric Research Education and Clinical Center, Bedford VA Medical Center, Bedford, MA 01730, USA
Biochim Biophys Acta 1762:373-80. 2006..These data suggest that combined minocycline and CoQ10 treatment may offer therapeutic benefit to patients suffering from HD... - Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic miceAlpaslan Dedeoglu
Geriatric Research Education and Clinical Center, Bedford VA Medical Center, Bedford, Massachussets 01730, USA
J Neurochem 85:1359-67. 2003..These findings have important therapeutic implications, suggesting that creatine therapy initiated after diagnosis may provide significant clinical benefits to HD patients... - Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic miceEdward C Stack
Geriatric Research Education and Clinical Center, Bedford Veterans Administration Medical Center, Bedford, Massachusetts 01730, USA
J Comp Neurol 490:354-70. 2005.... - Antioxidants modulate mitochondrial PKA and increase CREB binding to D-loop DNA of the mitochondrial genome in neuronsHoon Ryu
Geriatric Research Education and Clinical Center, Veteran s Affairs Medical Center, Bedford, MA 01730, USA
Proc Natl Acad Sci U S A 102:13915-20. 2005..These results suggest that the regulation of mitochondrial function via the mitochondrial PKA and CREB pathways may underlie some of the salutary effects of DFO in neurons... - ESET/SETDB1 gene expression and histone H3 (K9) trimethylation in Huntington's diseaseHoon Ryu
Geriatric Research Education and Clinical Center, Bedford Veteran s Affairs Medical Center, Bedford, MA 01730, USA
Proc Natl Acad Sci U S A 103:19176-81. 2006..Our data suggest that modulation of gene silencing mechanisms, through regulation of the ESET gene is important to neuronal survival and, as such, may be a promising treatment in HD patients... - Activation of Ets-2 by oxidative stress induces Bcl-xL expression and accounts for glial survival in amyotrophic lateral sclerosisJunghee Lee
Department of Neurology, Boston University School of Medicine, Boston, Massachusetts, USA
FASEB J 23:1739-49. 2009..This survival pathway may contribute to the glial survival and activation seen in the spinal cord of ALS patients... - Cytochrome C and caspase-9 expression in Huntington's diseaseTamara Kiechle
Geriatric Research Education and Clinical Center, Bedford VA Medical Center, MA 01730, USA
Neuromolecular Med 1:183-95. 2002.... - Increased 3-nitrotyrosine and oxidative damage in mice with a human copper/zinc superoxide dismutase mutationR J Ferrante
Geriatric Research Education Clinical Center, VA Medical Center, Bedford, MA, USA
Ann Neurol 42:326-34. 1997..These results suggest that the gain-of-function of at least one mutant SOD1 associated with FALS involves increased protein nitration and oxidative damage, which may play a role in neuronal degeneration... - MPTP induces alpha-synuclein aggregation in the substantia nigra of baboonsN W Kowall
Geriatric Research Education Clinical Center, Veterans Affairs Medical Center, Bedford, MA 01730, USA
Neuroreport 11:211-3. 2000..alpha-Synuclein aggregation induced by MPTP models the early stages of Lewy body formation and may be a fundamental step in the evolution of neuronal degeneration in PD... - Role of cyclooxygenase-2 induction by transcription factor Sp1 and Sp3 in neuronal oxidative and DNA damage responseJunghee Lee
Geriatric Research Education and Clinical Center, Bedford Veteran s Affairs Medical Center, 200 Springs Rd, Bedford, MA 01730, USA
FASEB J 20:2375-7. 2006..These results indicate that in primary neurons Sp1 and Sp3 play an essential role in the modulation of COX-2 transcription, which mediates neuronal homeostasis and survival by preventing DNA damage in response to neuronal stress... - Translational therapeutic strategies in amyotrophic lateral sclerosisHoon Ryu
Experimental Neuropathology Unit and Translational Therapeutics Laboratory, GRECC Unit 182B, Bedford VA Medical Center, 200 Springs Road, Bedford, MA 01730, USA
Mini Rev Med Chem 7:141-50. 2007..As in other neurodegenerative diseases, the most effective neuroprotection may result from combined treatment strategies... - Reduced creatine kinase as a central and peripheral biomarker in Huntington's diseaseJinho Kim
Geriatric Research Education Clinical Center, New England Veterans Administration VISN 1, Bedford, MA 01730, USA
Biochim Biophys Acta 1802:673-81. 2010.... - Neuroprotective effects of synaptic modulation in Huntington's disease R6/2 miceEdward C Stack
Geriatric Research Education and Clinical Center, Bedford Veterans Administration Medical Center, Bedford, Massachusetts 01730, USA
J Neurosci 27:12908-15. 2007..Thus, modulation of synaptic influences continues to have therapeutic potential in HD... 
Evidence of oxidant damage in Huntington's disease: translational strategies using antioxidantsEdward C Stack
Boston University School of Medicine and the Veterans Administration, Bedford, MA, USA
Ann N Y Acad Sci 1147:79-92. 2008..It is likely that high-dose coenzyme Q(10), creatine, or both agents, will represent a cornerstone defense in ameliorating the progression of HD...- Emerging chemotherapeutic strategies for Huntington's diseaseHoon Ryu
Boston University School of Medicine, Edith Nourse Rogers Veterans Administration Medical Center, Bedford, Massachusetts 01730, USA
Expert Opin Emerg Drugs 10:345-63. 2005..These compounds are discussed herein... - The therapeutic role of creatine in Huntington's diseaseHoon Ryu
Experimental Neuropathology Unit and Translational Therapeutics Laboratory, Geriatric Research Education Clinical Center, Bedford VA Medical Center, MA 01730, USA
Pharmacol Ther 108:193-207. 2005.... - Mutant SOD1G93A in bone marrow-derived cells exacerbates 3-nitropropionic acid induced striatal damage in miceQing Yuan Huang
Department of Neurology, Boston University School of Medicine, 715 Albany Street, E301 Boston, MA, USA
Neurosci Lett 418:175-80. 2007..These results demonstrate that altered SOD1 activity (mSOD(G93A)) in BMDCs affects striatal damage probably through a mechanism involving a systemic factor... - Mice overexpressing 70-kDa heat shock protein show increased resistance to malonate and 3-nitropropionic acidAlpaslan Dedeoglu
Department of Neurology, Boston University School of Medicine, Boston, Massachusetts, USA
Exp Neurol 176:262-5. 2002..These findings support the idea that HSP-70 has a neuroprotective role that may be useful in the treatment of neurodegenerative diseases... - Huntington's disease of the endocrine pancreas: insulin deficiency and diabetes mellitus due to impaired insulin gene expressionOle A Andreassen
Neurochemistry Laboratory, Massachusetts General Hospital, Boston, Massachusetts 02114, USA
Neurobiol Dis 11:410-24. 2002.... - Sequential activation of individual caspases, and of alterations in Bcl-2 proapoptotic signals in a mouse model of Huntington's diseaseYu Zhang
Neuroapoptosis Laboratory, Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115, USA
J Neurochem 87:1184-92. 2003..Understanding the chronology of apoptotic events provides important information for appropriate therapeutic targeting in this devastating and untreatable disease... - 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 dosageAlexandre Kuhn
Ecole Polytechnique Federale de Lausanne EPFL, 1015 Lausanne, Switzerland
Hum Mol Genet 16:1845-61. 2007.... - Nortriptyline delays disease onset in models of chronic neurodegenerationHongyan Wang
Neuroapoptosis Laboratory, Department of Neurosurgery, Brigham and Women s Hospital, Harvard Medical School, 221 Longwood Avenue, LMRC 113, Boston, MA 02115, USA
Eur J Neurosci 26:633-41. 2007..In addition, our data suggested a role for mPT in chronic neurodegeneration, particularly at the early rather than the advanced disease stages... - Monoallele deletion of CBP leads to pericentromeric heterochromatin condensation through ESET expression and histone H3 (K9) methylationJunghee Lee
Department of Neurology, Boston University School of Medicine, Boston, MA 02118, USA
Hum Mol Genet 17:1774-82. 2008..These results establish an alternative pathway that loss of CBP leads to the pericentric heterochromatin condensation through ESET expression and trimethylation of H3 (K9)... - The neuroprotective role of creatineAutumn M Klein
Neurology Department, Brigham and Womens Hospital, Boston, MA 02115, USA and Harvard Medical School, Boston, MA 02115, USA
Subcell Biochem 46:205-43. 2007.... - Inhibitors of cytochrome c release with therapeutic potential for Huntington's diseaseXin Wang
Neuroapoptosis Laboratory and Department of Neurosurgery, Brigham and Women s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
J Neurosci 28:9473-85. 2008..Given that many of these compounds have been approved by the Food and Drug Administration for clinical usage and cross the blood-brain barrier, these drugs may lead to trials in patients... - Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in miceShan Zhu
Neuroapoptosis Laboratory, Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
Nature 417:74-8. 2002..Because of the safety record of minocycline, and its ability to penetrate the blood-brain barrier, this drug may be a novel therapy for ALS... - Cdc42-interacting protein 4 binds to huntingtin: neuropathologic and biological evidence for a role in Huntington's diseaseSebastien Holbert
Laboratory of Genomic Biology, Institut National de la Santé et de la Recherche Médicale Avenir Group and Fondation Jean Dausset Centre d Etude du Polymorphisme Humain, 75010 Paris, France
Proc Natl Acad Sci U S A 100:2712-7. 2003..The overexpression of CIP4 induced the death of striatal neurons. Our data suggest that CIP4 accumulation and cellular toxicity may have a role in HD pathogenesis... - Sp1 is up-regulated in cellular and transgenic models of Huntington disease, and its reduction is neuroprotectiveZhihua Qiu
Massachusetts General Institute for Neurodegenerative Disease, Department of Neurology, Massachusetts General Hospital, Harvard Medical School, 114 16th Street, Charlestown, MA 02129, USA
J Biol Chem 281:16672-80. 2006..Our data suggest that enhancement of transcription factor Sp1 contributes to the pathology of HD and demonstrates that its suppression is beneficial... - Cystamine increases L-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregationJonathan H Fox
MassGeneral Institute for Neurodegenerative Disease, Department of Neurology, Massachusetts General Hospital, Charlestown, Massachusetts, USA
J Neurochem 91:413-22. 2004..We suggest that cystamine's neuroprotective effect in HD transgenic mice results from pleiotropic effects that include transglutaminase inhibition and antioxidant activity... - Experimental therapeutics in transgenic mouse models of Huntington's diseaseM Flint Beal
Department of Neurology and Neuroscience, Weill Medical College of Cornell University, Room F610, 525 East 68th Street, New York, NY 10021, USA
Nat Rev Neurosci 5:373-84. 2004 - Depletion of wild-type huntingtin in mouse models of neurologic diseasesYu Zhang
Neuroapoptosis Laboratory, Department of Neurosurgery, Brigham and Women s Hospital, Harvard Medical School, Boston, Massachusetts, USA
J Neurochem 87:101-6. 2003..We propose that in HD, in addition to a toxic gain-of-function of mutant huntingtin, a parallel depletion of wild-type huntingtin results in a detrimental loss-of-function, playing an important role in disease progression... - Anti-inflammatory treatment with acetylsalicylate or rofecoxib is not neuroprotective in Huntington's disease transgenic miceFran Norflus
MassGeneral Institute for Neurodegenerative Disease, Massachusetts General Hospital, Charlestown, MA 02129, USA
Neurobiol Dis 17:319-25. 2004..The studies showed that neither drug benefited the animals at doses comparable to those tolerated by humans... - Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's diseaseGabriella Gardian
Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York-Presbyterian Hospital, New York, New York 10021, USA
J Biol Chem 280:556-63. 2005.... - Minocycline inhibits caspase-independent and -dependent mitochondrial cell death pathways in models of Huntington's diseaseXin Wang
Neuroapoptosis Laboratory, Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115, USA
Proc Natl Acad Sci U S A 100:10483-7. 2003..Our results further delineate the mechanism by which minocycline mediates its remarkably broad neuroprotective effects... - Therapeutic attenuation of mitochondrial dysfunction and oxidative stress in neurotoxin models of Parkinson's diseaseEdward C Stack
Department of Neurology, Boston University School of Medicine, Boston, MA 02118, USA
Biochim Biophys Acta 1782:151-62. 2008..Together these findings show cystamine's therapeutic benefit to reduce neuronal loss through attenuation of oxidative stress and mitochondrial dysfunction, providing the rationale for human clinical trials in PD patients... - Translating therapies for Huntington's disease from genetic animal models to clinical trialsSteven M Hersch
Neurology Service, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts 02114, USA
NeuroRx 1:298-306. 2004.... - Increased survival and neuroprotective effects of BN82451 in a transgenic mouse model of Huntington's diseasePeter Klivenyi
Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York-Presbyterian Hospital, New York 10021, USA
J Neurochem 86:267-72. 2003..These findings provide evidence that novel anti-oxidants such as BN82451 may be useful for treating HD... - Sp1 and Sp3 are oxidative stress-inducible, antideath transcription factors in cortical neuronsHoon Ryu
Department of Neurology, Harvard Medical School, Boston, Massachusetts 02115, USA
J Neurosci 23:3597-606. 2003..Taken together, these results establish Sp1 and Sp3 as oxidative stress-induced transcription factors in cortical neurons that positively regulate neuronal survival... - 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 diseaseBruce G Jenkins
MGH NMR Center, Department of Radiology, Massachusetts General Hospital and Harvard Medical School, Charlestown, Massachusetts 02129, USA
J Neurochem 95:553-62. 2005..Our results indicate profound metabolic defects that are strongly affected by CAG repeat length, as well as gene expression levels and protein context... - Histone deacetylase inhibitors prevent oxidative neuronal death independent of expanded polyglutamine repeats via an Sp1-dependent pathwayHoon Ryu
Department of Neurology and Program in Neuroscience, Harvard Medical School and Beth Israel Deaconess Medical Center, Harvard Institutes of Medicine, Boston, MA 02115, USA
Proc Natl Acad Sci U S A 100:4281-6. 2003..Together, these results demonstrate that HDAC inhibitors inhibit oxidative death independent of polyglutamine expansions by activating an Sp1-dependent adaptive response... - Mice lacking alpha-synuclein are resistant to mitochondrial toxinsPeter Klivenyi
Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, 525 East 68th Street, New York, NY 10021, USA
Neurobiol Dis 21:541-8. 2006..These findings implicate alpha-synuclein as a modulator of oxidative damage, which has been implicated in neuronal death produced by MPTP and other mitochondrial toxins... - Mitochondrial cyclic AMP response element-binding protein (CREB) mediates mitochondrial gene expression and neuronal survivalJunghee Lee
Neurology Department, Boston University School of Medicine, Boston, Massachusetts 02118, USA
J Biol Chem 280:40398-401. 2005.... - Transcriptional therapy with the histone deacetylase inhibitor trichostatin A ameliorates experimental autoimmune encephalomyelitisSandra Camelo
Laboratory of Transcriptional and Immune Regulation, Brigham and Women's Hospital, Department of Neurology, Harvard Medical School, Boston, MA 02139, USA
J Neuroimmunol 164:10-21. 2005..A transcriptional imbalance in MS may contribute to immune dysregulation and neurodegeneration, and we identify HDAC inhibition as a transcriptional intervention to ameliorate this imbalance... - Genetic and pharmacological inactivation of the adenosine A2A receptor attenuates 3-nitropropionic acid-induced striatal damageJ Stephen Fink
Department of Neurology, Boston University School of Medicine, MA 02118, USA
J Neurochem 88:538-44. 2004.... - Prophylactic creatine administration mediates neuroprotection in cerebral ischemia in miceShan Zhu
Neuroapoptosis Laboratory, Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
J Neurosci 24:5909-12. 2004..Prophylactic creatine supplementation, similar to what is recommended for an agent such as aspirin, may be considered for patients in high stroke-risk categories... - Conformation-sensitive antibodies against alzheimer amyloid-beta by immunization with a thioredoxin-constrained B-cell epitope peptideNadia Moretto
Department of Biochemistry and Molecular Biology, Chiesi Farmaceutici, University of Parma, 43100 Parma, Italy
J Biol Chem 282:11436-45. 2007..The data indicate that a conformational epitope shared by oligomers and fibrils can be mimicked by a thioredoxin-constrained Abeta fragment repeat and identify Trx(Abeta15)4 as a promising new tool for AD immunotherapy... - Huntington's disease: progress and potential in the fieldEdward C Stack
Boston University School of Medicine, Department of Neurology, Boston, Massachusetts, MA 20118, USA
Expert Opin Investig Drugs 16:1933-53. 2007..This review focuses on these studies and the compounds that hold promise for treating human Huntington's disease... - Mitochondrial nuclear receptors and transcription factors: who's minding the cell?Junghee Lee
Department of Neurology, Boston University School of Medicine, Boston, Massachusetts, USA
J Neurosci Res 86:961-71. 2008..Thus, the modulation of signaling pathways via mitochondria-targeting nuclear receptors and transcription factors is rapidly emerging as a novel therapeutic target...
Research Grants
- OXIDATIVE INJURY IN PARKINSONS DISEASEROBERT FERRANTE; Fiscal Year: 2002..These studies will have direct relevance to understanding the pathogenesis of PD and to developing new therapies. ..
- Combination Drug Therapy in Huntington's DiseaseROBERT FERRANTE; Fiscal Year: 2007..abstract_text> ..