M F Beal

Summary

Affiliation: Cornell University
Country: USA

Publications

  1. pmc Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation
    Rajnish K Chaturvedi
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY 10065, USA
    Hum Mol Genet 19:3190-205. 2010
  2. ncbi request reprint Oxidative metabolism
    M F Beal
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University and New York Presbyterian Hospital, 525 East 68th Street, Room F610, New York, New York 10021, USA
    Ann N Y Acad Sci 924:164-9. 2000
  3. ncbi request reprint Oxidative damage as an early marker of Alzheimer's disease and mild cognitive impairment
    M Flint Beal
    Neurology Department, Weill Medical College of Cornell University, 525 East 68th Street, Suite F610, New York, NY 10021, USA
    Neurobiol Aging 26:585-6. 2005
  4. ncbi request reprint Commentary on "Alpha-synuclein and mitochondria: a tangled skein"
    M Flint Beal
    Weill Medical College of Cornell University, New York, NY 10021, USA
    Exp Neurol 186:109-11. 2004
  5. doi request reprint Therapeutic approaches to mitochondrial dysfunction in Parkinson's disease
    M Flint Beal
    Department of Neurology and Neuroscience, Weill Cornell Medical College, New York, NY 10065 4870, USA
    Parkinsonism Relat Disord 15:S189-94. 2009
  6. ncbi request reprint Oxidatively modified proteins in aging and disease
    M Flint Beal
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, NY 10021, USA
    Free Radic Biol Med 32:797-803. 2002
  7. ncbi request reprint Coenzyme Q10 as a possible treatment for neurodegenerative diseases
    M Flint Beal
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, NY 10021, USA
    Free Radic Res 36:455-60. 2002
  8. ncbi request reprint Bioenergetic approaches for neuroprotection in Parkinson's disease
    M Flint Beal
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, NY, USA
    Ann Neurol 53:S39-47; discussion S47-8. 2003
  9. ncbi request reprint Experimental models of Parkinson's disease
    M F Beal
    Department of Neurology, New York Hospital Cornell Medical Center, 525 East 68th Street, New York, New York 10021, USA
    Nat Rev Neurosci 2:325-34. 2001
  10. ncbi request reprint Mice deficient in dihydrolipoamide dehydrogenase show increased vulnerability to MPTP, malonate and 3-nitropropionic acid neurotoxicity
    Peter Klivenyi
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York 10021, USA
    J Neurochem 88:1352-60. 2004

Collaborators

Detail Information

Publications125 found, 100 shown here

  1. pmc Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation
    Rajnish K Chaturvedi
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY 10065, USA
    Hum Mol Genet 19:3190-205. 2010
    ....
  2. ncbi request reprint Oxidative metabolism
    M F Beal
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University and New York Presbyterian Hospital, 525 East 68th Street, Room F610, New York, New York 10021, USA
    Ann N Y Acad Sci 924:164-9. 2000
    ..It is therefore possible that therapeutic strategies to improve brain metabolism or ameliorate oxidative damage might be useful in treating Alzheimer's disease...
  3. ncbi request reprint Oxidative damage as an early marker of Alzheimer's disease and mild cognitive impairment
    M Flint Beal
    Neurology Department, Weill Medical College of Cornell University, 525 East 68th Street, Suite F610, New York, NY 10021, USA
    Neurobiol Aging 26:585-6. 2005
  4. ncbi request reprint Commentary on "Alpha-synuclein and mitochondria: a tangled skein"
    M Flint Beal
    Weill Medical College of Cornell University, New York, NY 10021, USA
    Exp Neurol 186:109-11. 2004
  5. doi request reprint Therapeutic approaches to mitochondrial dysfunction in Parkinson's disease
    M Flint Beal
    Department of Neurology and Neuroscience, Weill Cornell Medical College, New York, NY 10065 4870, USA
    Parkinsonism Relat Disord 15:S189-94. 2009
    ..Lastly, newly identified therapeutic targets include peroxisomal proliferator activated receptor gamma-coactivator (PGC-1alpha) and sirtuins. These pathways provide promise for future therapeutic developments in the treatment of PD...
  6. ncbi request reprint Oxidatively modified proteins in aging and disease
    M Flint Beal
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, NY 10021, USA
    Free Radic Biol Med 32:797-803. 2002
    ..These findings help to provide a rationale for trials of antioxidants in neurodegenerative diseases...
  7. ncbi request reprint Coenzyme Q10 as a possible treatment for neurodegenerative diseases
    M Flint Beal
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, NY 10021, USA
    Free Radic Res 36:455-60. 2002
    ..CoQ10 is presently being studied as a potential treatment for early PD as well as in combination with remacemide as a potential treatment for HD...
  8. ncbi request reprint Bioenergetic approaches for neuroprotection in Parkinson's disease
    M Flint Beal
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, NY, USA
    Ann Neurol 53:S39-47; discussion S47-8. 2003
    ..Many other agents show good human tolerability. These agents therefore are promising candidates for further study as neuroprotective agents in PD...
  9. ncbi request reprint Experimental models of Parkinson's disease
    M F Beal
    Department of Neurology, New York Hospital Cornell Medical Center, 525 East 68th Street, New York, New York 10021, USA
    Nat Rev Neurosci 2:325-34. 2001
    ..Here, I discuss the merits and limitations of these different animal models in our attempts to understand the physiology of Parkinson's disease and to develop new therapies...
  10. ncbi request reprint Mice deficient in dihydrolipoamide dehydrogenase show increased vulnerability to MPTP, malonate and 3-nitropropionic acid neurotoxicity
    Peter Klivenyi
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York 10021, USA
    J Neurochem 88:1352-60. 2004
    ..KGDHC activity was also found to be reduced in putamen from patients with HD. These findings provide further evidence that mitochondrial defects may contribute to the pathogenesis of neurodegenerative diseases...
  11. ncbi request reprint Oxidative stress in Huntington's disease
    S E Browne
    Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston 02114, USA
    Brain Pathol 9:147-63. 1999
    ..Here we review the evidence for oxidative damage and potential mechanisms of neuronal death in HD...
  12. ncbi request reprint Iron porphyrin treatment extends survival in a transgenic animal model of amyotrophic lateral sclerosis
    Annie S Wu
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York 10021, USA
    J Neurochem 85:142-50. 2003
    ..These results therefore provide further evidence of oxidative damage in a mouse model of ALS, and suggest that FeTCPP could be beneficial for the treatment of ALS patients...
  13. pmc Sensitivity to oxidative stress in DJ-1-deficient dopamine neurons: an ES- derived cell model of primary Parkinsonism
    Cecile Martinat
    Department of Pathology, Center for Neurobiology and Behavior, and Taub Institute, Columbia University, New York, New York, USA
    PLoS Biol 2:e327. 2004
    ..These data are consistent with a protective role for DJ-1, and demonstrate the utility of genetically modified embryonic stem cell-derived neurons as cellular models of neuronal disorders...
  14. pmc Combination therapy with coenzyme Q10 and creatine produces additive neuroprotective effects in models of Parkinson's and Huntington's diseases
    Lichuan Yang
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, New York 10021, USA
    J Neurochem 109:1427-39. 2009
    ..These findings suggest that combination therapy using CoQ(10) and creatine may be useful in the treatment of neurodegenerative diseases such as Parkinson's disease and HD...
  15. ncbi request reprint Further evidence for mitochondrial dysfunction in progressive supranuclear palsy
    D S Albers
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, New York 10021, USA
    Exp Neurol 168:196-8. 2001
    ....
  16. ncbi request reprint Frontal lobe dysfunction in progressive supranuclear palsy: evidence for oxidative stress and mitochondrial impairment
    D S Albers
    Department of Neurology and Neuroscience, Cornell University Medical College, New York, NY 10021, USA
    J Neurochem 74:878-81. 2000
    ..Together, these results suggest that mitochondrial dysfunction and lipid peroxidation may underlie the frontal metabolic and functional deficits observed in PSP...
  17. ncbi request reprint Mitochondrial alpha-ketoglutarate dehydrogenase complex generates reactive oxygen species
    Anatoly A Starkov
    Department of Neurology and Neuroscience, Weill Medical College, Cornell University, New York, New York 10021, USA
    J Neurosci 24:7779-88. 2004
    ..These mitochondria also produced significantly less H(2)O(2) than mitochondria isolated from their littermate wild-type mice. The data strongly indicate that KGDHC is a primary site of ROS production in normally functioning mitochondria...
  18. ncbi request reprint Matrix metalloproteinase-9 is elevated in 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-induced parkinsonism in mice
    Stefan Lorenzl
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY, USA
    Neuromolecular Med 5:119-32. 2004
    ..These results indicate that MMP-9 is induced after MPTP application in mice and that pharmacologic inhibition of MMPs protects against MPTP neurotoxicity...
  19. ncbi request reprint Additive neuroprotective effects of creatine and a cyclooxygenase 2 inhibitor against dopamine depletion in the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) mouse model of Parkinson's disease
    Peter Klivenyi
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, NY 10021, USA
    J Mol Neurosci 21:191-8. 2003
    ..These results suggest that a combination of a COX-2 inhibitor with creatine might be a useful neuroprotective strategy for PD...
  20. ncbi request reprint Therapeutic effects of coenzyme Q10 (CoQ10) and reduced CoQ10 in the MPTP model of Parkinsonism
    Carine Cleren
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, New York 10021, USA
    J Neurochem 104:1613-21. 2008
    ..These results provide further evidence that administration of CoQ10 is a promising therapeutic strategy for the treatment of PD...
  21. ncbi request reprint Cell-permeable peptide antioxidants as a novel therapeutic approach in a mouse model of amyotrophic lateral sclerosis
    Susanne Petri
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY, USA
    J Neurochem 98:1141-8. 2006
    ..This further enhances the concept that pharmacological modification of oxidative stress is a therapeutic option for the treatment of ALS...
  22. doi request reprint Lenalidomide (Revlimid) administration at symptom onset is neuroprotective in a mouse model of amyotrophic lateral sclerosis
    Arie Neymotin
    Department of Neurology, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, NY, USA
    Exp Neurol 220:191-7. 2009
    ..These data encourage further clinical evaluation of lenalidomide as therapeutic strategy to block or slow disease progression in human ALS patients...
  23. ncbi request reprint Mitochondrial impairment in the cerebellum of the patients with progressive supranuclear palsy
    L C Park
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 785 Mamaroneck Avenue, White Plains, NY 10605, USA
    J Neurosci Res 66:1028-34. 2001
    ....
  24. pmc Thiamine deficiency induces oxidative stress and exacerbates the plaque pathology in Alzheimer's mouse model
    Saravanan S Karuppagounder
    Department of Neurology and Neurosciences, Weill Medical College of Cornell University, Burke Medical Research Institute, 785 Mamaroneck Avenue, White Plains, NY 10605, USA
    Neurobiol Aging 30:1587-600. 2009
    ..Thus, the induction of mild impairment of oxidative metabolism, oxidative stress and inflammation induced by TD alters metabolism of APP and/or Abeta and promotes accumulation of plaques independent of neuron loss or neuritic clusters...
  25. ncbi request reprint Minocycline enhances MPTP toxicity to dopaminergic neurons
    Lichuan Yang
    Neurochemistry and Neurodegenerative Disease Laboratory, Weill Medical College at Cornell University, New York, New York 10021, USA
    J Neurosci Res 74:278-85. 2003
    ..We present evidence suggesting that this effect may be due to inhibition of DA and 1-methyl-4-phenylpridium (MPP+) uptake into striatal vesicles...
  26. ncbi request reprint Increased survival and neuroprotective effects of BN82451 in a transgenic mouse model of Huntington's disease
    Peter 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...
  27. pmc Mutant LRRK2(R1441G) BAC transgenic mice recapitulate cardinal features of Parkinson's disease
    Yanping Li
    Department of Neurology and Neurosciences, Weill Medical College of Cornell University, New York, New York, USA
    Nat Neurosci 12:826-8. 2009
    ..These mice provide a valid model of Parkinson's disease and are a resource for the investigation of pathogenesis and therapeutics...
  28. ncbi request reprint Neuroprotective mechanisms of creatine occur in the absence of mitochondrial creatine kinase
    Peter Klivenyi
    Department of Neurology and Neuroscience, New York Presbyterian Hospital, Weill Medical College of Cornell University, New York, NY 10021, USA
    Neurobiol Dis 15:610-7. 2004
    ....
  29. ncbi request reprint Celastrol protects against MPTP- and 3-nitropropionic acid-induced neurotoxicity
    Carine Cleren
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, New York, USA
    J Neurochem 94:995-1004. 2005
    ..Celastrol is therefore a promising neuroprotective agent for the treatment of PD and HD...
  30. ncbi request reprint Somatic mitochondrial DNA mutations in single neurons and glia
    Ippolita Cantuti-Castelvetri
    Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02129, USA
    Neurobiol Aging 26:1343-55. 2005
    ..These mutations may contribute to changes in brain function during normal aging and neurodegenerative disorders...
  31. ncbi request reprint Low mutational burden of individual acquired mitochondrial DNA mutations in brain
    D K Simon
    Department of Neurology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts 02115, USA
    Genomics 73:113-6. 2001
    ..This does not exclude the possibility that the cumulative burden of multiple, individually rare, acquired mutations impairs mitochondrial function...
  32. ncbi request reprint Neuroprotective effects of phenylbutyrate against MPTP neurotoxicity
    Gabriella Gardian
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, 525 East 68th Street, Room F610, New York, NY 10021, USA
    Neuromolecular Med 5:235-41. 2004
    ..These findings provide further evidence that administration of phenylbutyrate may be a useful approach for the treatment of neurodegenerative diseases...
  33. ncbi request reprint The lipophilic metal chelators DP-109 and DP-460 are neuroprotective in a transgenic mouse model of amyotrophic lateral sclerosis
    Susanne Petri
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, New York, USA
    J Neurochem 102:991-1000. 2007
    ..In line with previous studies using metal chelators in the G93A animal model, our results suggest that these compounds have neuroprotective capacities in ALS...
  34. ncbi request reprint Somatic mitochondrial DNA mutations in cortex and substantia nigra in aging and Parkinson's disease
    David K Simon
    Department of Neurology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02115, USA
    Neurobiol Aging 25:71-81. 2004
    ..These results indicate that individually rare mtDNA point mutations reach a high aggregate burden in FCtx and SN of elderly subjects...
  35. ncbi request reprint Additive neuroprotective effects of creatine and cyclooxygenase 2 inhibitors in a transgenic mouse model of amyotrophic lateral sclerosis
    Peter Klivenyi
    Department of Neurology and Neuroscience, New York Presbyterian Hospital Weill Medical College of Cornell University, New York, New York 10021, USA
    J Neurochem 88:576-82. 2004
    ..These results suggest that combinations of therapies targeting different disease mechanisms may be a useful strategy in the treatment of ALS...
  36. ncbi request reprint Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients
    Falk R Wiedemann
    Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, USA
    J Neurochem 80:616-25. 2002
    ....
  37. ncbi request reprint Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease
    Gabriella 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
    ....
  38. pmc Mice deficient in dihydrolipoyl succinyl transferase show increased vulnerability to mitochondrial toxins
    Lichuan Yang
    Department of Neurology and Neuroscience, Weill Cornell Medical College, New York, NY 10065, USA
    Neurobiol Dis 36:320-30. 2009
    ..These observations support the hypothesis that reductions in KGDHC may impair the adaptability of the brain and contribute to the pathogenesis of neurodegenerative diseases...
  39. ncbi request reprint Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease
    M B Bogdanov
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University and New York Presbyterian Hospital, New York, NY 10021, USA
    J Neurochem 79:1246-9. 2001
    ..Immunocytochemistry showed increased OH(8)dG staining in late stages of the illness. These results suggest that oxidative damage may play a role in the pathogenesis of neuronal degeneration in the R6/2 transgenic mouse model of HD...
  40. ncbi request reprint Treatment with a catalytic antioxidant corrects the neurobehavioral defect in ataxia-telangiectasia mice
    Susan E Browne
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY, USA
    Free Radic Biol Med 36:938-42. 2004
    ..We show that treatment with a catalytic antioxidant corrects the neurobehavioral deficit in these mice...
  41. pmc Creatine and its potential therapeutic value for targeting cellular energy impairment in neurodegenerative diseases
    Peter J Adhihetty
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 525 East 68th Street, New York, NY 10021, USA
    Neuromolecular Med 10:275-90. 2008
    ..In summary, current literature suggests that exogenous creatine supplementation is most efficacious as a treatment paradigm in Huntington's and Parkinson's disease but appears to be less effective for ALS and Alzheimer's disease...
  42. pmc Intraneuronal Alzheimer abeta42 accumulates in multivesicular bodies and is associated with synaptic pathology
    Reisuke H Takahashi
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, New York 10021, USA
    Am J Pathol 161:1869-79. 2002
    ..This accumulation was associated with abnormal synaptic morphology, before beta-amyloid plaque pathology, suggesting that intracellular accumulation of beta-amyloid plays a crucial role in Alzheimer's disease...
  43. ncbi request reprint A pivotal role of matrix metalloproteinase-3 activity in dopaminergic neuronal degeneration via microglial activation
    Yoon Seong Kim
    Burke Medical Research Institute, and Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 1300 York Ave, New York, NY 10021, USA
    FASEB J 21:179-87. 2007
    ..Our results could lead to a novel therapeutic approach to PD...
  44. pmc Reduction of oxidative stress, amyloid deposition, and memory deficit by manganese superoxide dismutase overexpression in a transgenic mouse model of Alzheimer's disease
    Magali Dumont
    Weill Cornell Medical College, Department of Neurology and Neuroscience, 525 E 68th St, New York, NY 10065, USA
    FASEB J 23:2459-66. 2009
    ....
  45. ncbi request reprint Stimulation of beta-amyloid precursor protein trafficking by insulin reduces intraneuronal beta-amyloid and requires mitogen-activated protein kinase signaling
    L Gasparini
    Laboratory of Molecular and Cellular Neuroscience, Fisher Center for Research on Alzheimer Disease, and Laboratory for Mass Spectrometry, The Rockefeller University, New York, New York 10021, USA
    J Neurosci 21:2561-70. 2001
    ..The results suggest cell biological and signal transduction mechanisms by which insulin modulates betaAPP and Abeta trafficking in neuronal cultures...
  46. ncbi request reprint The role of mitochondria in the pathogenesis of neurodegenerative diseases
    G Manfredi
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University and the New York Hospital, Cornell Medical Center, New York 10021, USA
    Brain Pathol 10:462-72. 2000
    ..In either case, understanding the role of mitochondria in the pathogenesis of neurodegenerative diseases could be important for the development of therapeutic strategies in these disorders...
  47. ncbi request reprint Mitochondrial DNA mutations in complex I and tRNA genes in Parkinson's disease
    D K Simon
    Department of Neurology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02115, USA
    Neurology 54:703-9. 2000
    ..To identify mitochondrial DNA (mtDNA) mutations that predispose to PD...
  48. pmc Protection from Alzheimer's-like disease in the mouse by genetic ablation of inducible nitric oxide synthase
    Carl Nathan
    Department of Microbiology and Immunology, Weill Cornell Medical College, New York, NY 10021, USA
    J Exp Med 202:1163-9. 2005
    ..Thus, iNOS seems to be a major instigator of beta-amyloid deposition and disease progression. Inhibition of iNOS may be a therapeutic option in AD...
  49. ncbi request reprint PGC-1alpha, a new therapeutic target in Huntington's disease?
    Jetta K McGill
    Department of Neurology, Cornell Medical Center, New York, NY 10021, USA
    Cell 127:465-8. 2006
    ....
  50. pmc Mitochondria targeted peptides protect against 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine neurotoxicity
    Lichuan Yang
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, New York 10021, USA
    Antioxid Redox Signal 11:2095-104. 2009
    ..These findings provide strong evidence that these neuroprotective peptides, which target both mitochondrial dysfunction and oxidative damage, are a promising approach for the treatment of PD...
  51. ncbi request reprint Stress-induced mitochondrial depolarization and oxidative damage in PSP cybrids
    Jason W Chirichigno
    Department of Neurology and Neuroscience, Room A 503, Weill Medical College of Cornell University, 525 East 68th Street, New York, NY 10021, USA
    Brain Res 951:31-5. 2002
    ....
  52. pmc Influence of mitochondrial enzyme deficiency on adult neurogenesis in mouse models of neurodegenerative diseases
    N Y Calingasan
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY 10065, USA
    Neuroscience 153:986-96. 2008
    ..Our findings support the view that mitochondrial dysfunction can influence the number of neural progenitor cells in the hippocampus of adult mice...
  53. ncbi request reprint Beta-amyloid 42 accumulation in the lumbar spinal cord motor neurons of amyotrophic lateral sclerosis patients
    Noel Y Calingasan
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 525 East 68th Street, F 610, New York, NY 10021, USA
    Neurobiol Dis 19:340-7. 2005
    ..These results suggest that aberrant accumulation of A beta42 in ALS spinal cord motor neurons is associated with oxidative stress, and may play a role in the pathogenesis of neurodegeneration in ALS...
  54. ncbi request reprint Integrative role of cPLA with COX-2 and the effect of non-steriodal anti-inflammatory drugs in a transgenic mouse model of amyotrophic lateral sclerosis
    Mahmoud Kiaei
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, New York 10021, USA
    J Neurochem 93:403-11. 2005
    ..These results suggest that cPLA2 plays an important role in supplying arachidonic acid to the COX-2 driven inflammatory pathway in ALS associated with SOD1 mutations...
  55. doi request reprint Mitochondrial biology and oxidative stress in Parkinson disease pathogenesis
    Claire Henchcliffe
    Department of Neurology and Neuroscience at the Weill Medical College of Cornell University, New York, NY 10021, USA
    Nat Clin Pract Neurol 4:600-9. 2008
    ....
  56. ncbi request reprint Neurotoxicity and behavioral deficits associated with Septin 5 accumulation in dopaminergic neurons
    Jin H Son
    Laboratory of Molecular Neurobiology, The W M Burke Medical Research Institute, White Plains, New York 10605, USA
    J Neurochem 94:1040-53. 2005
    ..These data suggest that a threshold level of Septin 5 accumulation is required for DAergic cell loss and that l-DOPA-responsive motor deficits can occur even in the presence of elevated DA...
  57. doi request reprint The urokinase system of plasminogen activator plays a role in amyotrophic lateral sclerosis (ALS) pathogenesis
    M Flint Beal
    Cornell University Medical College, Department of Neurology, 525 East 68th Street, New York, NY 10021, USA
    Exp Neurol 211:332-3. 2008
  58. ncbi request reprint Mitochondria take center stage in aging and neurodegeneration
    M Flint Beal
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, NY 10021, USA
    Ann Neurol 58:495-505. 2005
    ..Therapeutic approaches targeting mitochondrial dysfunction and oxidative damage in these diseases therefore have great promise...
  59. ncbi request reprint Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice
    Marina Mattiazzi
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, New York 10021, USA
    J Biol Chem 277:29626-33. 2002
    ..Our findings suggest that G93A-mutated hSOD1 in mitochondria may cause mitochondrial defects, which contribute to precipitating the neurodegenerative process in motor neurons...
  60. ncbi request reprint Mice lacking alpha-synuclein are resistant to mitochondrial toxins
    Peter 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...
  61. ncbi request reprint Mitochondrial dysfunction and amyotrophic lateral sclerosis
    Isabel Hervias
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 525 East 68th Street, A 505, New York, New York 10021, USA
    Muscle Nerve 33:598-608. 2006
    ..If mitochondrial dysfunction is convincingly involved in ALS pathogenesis, either as a primary cause or as contributing factor, it is likely to become a novel target for therapeutic intervention...
  62. ncbi request reprint Bioenergetic abnormalities in discrete cerebral motor pathways presage spinal cord pathology in the G93A SOD1 mouse model of ALS
    Susan E Browne
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 525 E 68th Street, A 502, New York, NY 10021, USA
    Neurobiol Dis 22:599-610. 2006
    ....
  63. ncbi request reprint Functional engraftment of human ES cell-derived dopaminergic neurons enriched by coculture with telomerase-immortalized midbrain astrocytes
    Neeta S Roy
    Department of Neurology, Weill Medical College of Cornell University, New York, New York 10021, USA
    Nat Med 12:1259-68. 2006
    ..Yet these data also mandate caution in the clinical application of HES cell-derived grafts, given their potential for phenotypic instability and undifferentiated expansion...
  64. ncbi request reprint Peroxisome proliferator-activated receptor-gamma agonist extends survival in transgenic mouse model of amyotrophic lateral sclerosis
    Mahmoud Kiaei
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, 525 East 68th Street, Room A 501, New York, NY 10021, USA
    Exp Neurol 191:331-6. 2005
    ..Pioglitazone also reduced iNOS, NFkappa-B, and 3-nitrotyrosine immunoreactivity in the spinal cords of G93A transgenic mice. These results suggest that pioglitazone may have therapeutic potential for human ALS...
  65. pmc The role of NADPH oxidase 1-derived reactive oxygen species in paraquat-mediated dopaminergic cell death
    Ana Clara Cristóvão
    Neurology Neuroscience Department, Weill Medical College of Cornell University, New York, New York, USA
    Antioxid Redox Signal 11:2105-18. 2009
    ..Our results suggest that Nox1-generated superoxide is implicated in the oxidative stress elicited by paraquat in DA cells, and it can serve as a novel target for pharmacologic intervention...
  66. pmc Impaired PGC-1alpha function in muscle in Huntington's disease
    Rajnish K Chaturvedi
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, NY 10065, USA
    Hum Mol Genet 18:3048-65. 2009
    ..Furthermore, muscle may provide a readily accessible tissue in which to monitor therapeutic interventions...
  67. doi request reprint Mitochondrial therapies for Parkinson's disease
    Bobby Thomas
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, New York 10065, USA
    Mov Disord 25:S155-60. 2010
    ..We also review novel mitochondrial survival pathways that provide hope and promise for innovative neuroprotective therapies in the future that can be explored as possible therapeutic intervention for PD pathogenesis...
  68. ncbi request reprint Therapeutic effects of coenzyme Q10 in neurodegenerative diseases
    M Flint Beal
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, New York 10021, USA
    Methods Enzymol 382:473-87. 2004
  69. doi request reprint Multinuclear magnetic resonance spectroscopy for in vivo assessment of mitochondrial dysfunction in Parkinson's disease
    Claire Henchcliffe
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY 10021, USA
    Ann N Y Acad Sci 1147:206-20. 2008
    ....
  70. pmc Behavioral deficits and progressive neuropathology in progranulin-deficient mice: a mouse model of frontotemporal dementia
    Fangfang Yin
    Department of Microbiology and Immunology, Weill Cornell Medical College, New York, NY 10065, USA
    FASEB J 24:4639-47. 2010
    ..Thus, progranulin deficiency induced FTD-like behavioral and neuropathological deficits. These mice may serve as an important tool for deciphering underlying mechanisms in frontotemporal dementia...
  71. pmc Cause and consequence: mitochondrial dysfunction initiates and propagates neuronal dysfunction, neuronal death and behavioral abnormalities in age-associated neurodegenerative diseases
    Gary E Gibson
    Department of Neurology and Neuroscience, Weill Cornell Medical College of Cornell University at Burke Medical Research Institute, 785 Mamaroneck Avenue, White Plains, NY 10605, USA
    Biochim Biophys Acta 1802:122-34. 2010
    ..Altogether, our results suggest that increasing KGDHC via inhibition of TGase or via a host of other strategies to be described would be effective therapeutic approaches in age-associated neurodegenerative diseases...
  72. pmc Mitochondrial dihydrolipoyl succinyltransferase deficiency accelerates amyloid pathology and memory deficit in a transgenic mouse model of amyloid deposition
    Magali Dumont
    Department of Neurology and Neuroscience, Weill Cornell Medical College, New York, NY 10065, USA
    Free Radic Biol Med 47:1019-27. 2009
    ..Our data suggest that alpha-KGDHC may be involved in AD pathogenesis through increased mitochondrial oxidative stress...
  73. ncbi request reprint Diffusion tensor imaging in the diagnosis of primary lateral sclerosis
    Aziz M Ulug
    Department of Radiology, Weill Medical College of Cornell University, New York, New York 10021, USA
    J Magn Reson Imaging 19:34-9. 2004
    ..To evaluate the utility of MR diffusion tensor imaging in diagnosing primary lateral sclerosis (PLS)...
  74. ncbi request reprint Promethazine protects against 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine neurotoxicity
    Carine Cleren
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 525 East 68th Street, Room A 501, NY 10021, USA
    Neurobiol Dis 20:701-8. 2005
    ..The sum of data indicates that PMZ is a strong neuroprotective agent capable of protecting dopaminergic neurons against MPTP toxicity in vivo...
  75. ncbi request reprint Mitochondria, oxidative damage, and inflammation in Parkinson's disease
    M Flint Beal
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, New York 10021, USA
    Ann N Y Acad Sci 991:120-31. 2003
    ..Of these, coenzyme Q(10) appears to be particularly promising based on the results of a recent phase 2 clinical trial in which it significantly slowed the progression of PD...
  76. ncbi request reprint Effects of Coenzyme Q10 in Huntington's disease and early Parkinson's disease
    M Flint Beal
    Department of Neurology and Neuroscience, New York Presbyterian Hospital, New York, NY 10021, USA
    Biofactors 18:153-61. 2003
  77. ncbi request reprint Parkinson's disease
    Bobby Thomas
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 525 East 68th Street, A 501, New York, NY 10021, USA
    Hum Mol Genet 16:R183-94. 2007
    ....
  78. ncbi request reprint Mitochondrial dysfunction in progressive supranuclear palsy
    David S Albers
    Department of Neurology and Neuroscience, Weill Medical College, Cornell University, Room A 503, 525 East 68th Street, New York, NY 10021, USA
    Neurochem Int 40:559-64. 2002
    ....
  79. ncbi request reprint Celastrol blocks neuronal cell death and extends life in transgenic mouse model of amyotrophic lateral sclerosis
    Mahmoud Kiaei
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, NY 10021, USA
    Neurodegener Dis 2:246-54. 2005
    ..Celastrol has been widely used in treating inflammatory diseases in man, and is well tolerated; therefore, it may be a promising therapeutic candidate for the treatment of human ALS...
  80. ncbi request reprint Thalidomide and lenalidomide extend survival in a transgenic mouse model of amyotrophic lateral sclerosis
    Mahmoud Kiaei
    Department of Neurology and Neuroscience, Weill Medical College, Cornell University, New York Presbyterian Hospital, New York, New York 10021, USA
    J Neurosci 26:2467-73. 2006
    ..Both compounds also reduced interleukin (IL)-12p40, IL-1alpha, and IL-1beta and increased IL-RA and TGF-beta1 mRNA. Therefore, both thalidomide and lenalidomide bear promise as therapeutic interventions for the treatment of ALS...
  81. pmc Pulse inhibition of histone deacetylases induces complete resistance to oxidative death in cortical neurons without toxicity and reveals a role for cytoplasmic p21(waf1/cip1) in cell cycle-independent neuroprotection
    Brett Langley
    Burke Medical Research Institute, White Plains, New York 10605, USA
    J Neurosci 28:163-76. 2008
    ....
  82. ncbi request reprint Urinary 8-hydroxy-2'-deoxyguanosine, a metabolite of oxidized DNA, is not elevated in HIV patients on combination antiretroviral therapy
    Simon Paul
    Department of Medicine, Division of Infectious Diseases, New York Presbyterian Hospital Weill College of Medicine of Cornell University, New York, NY 10021, USA
    Free Radic Res 37:499-502. 2003
    ..Free Radical Biology and Medicine 27 (1999) 647-666]...
  83. ncbi request reprint Additive neuroprotective effects of a histone deacetylase inhibitor and a catalytic antioxidant in a transgenic mouse model of amyotrophic lateral sclerosis
    Susanne Petri
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 525 East 68th Street, New York, NY 10021, USA
    Neurobiol Dis 22:40-9. 2006
    ....
  84. ncbi request reprint Mitochondrial dysfunction and oxidative damage in Alzheimer's and Parkinson's diseases and coenzyme Q10 as a potential treatment
    M Flint Beal
    Department of Neurology and Neuroscience, New York Presbyterian Hospital Weill Medical College of Cornell University, 525 East 68th Street, New York, New York 10021, USA
    J Bioenerg Biomembr 36:381-6. 2004
    ..Lastly, I reviewed the potential efficacy of coenzyme Q as well as a number of other antioxidants in the treatment of both Parkinson's and Alzheimer's diseases...
  85. ncbi request reprint The alpha-ketoglutarate-dehydrogenase complex: a mediator between mitochondria and oxidative stress in neurodegeneration
    Gary E Gibson
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY, USA
    Mol Neurobiol 31:43-63. 2005
    ..Studies of proteins, cells, animal models, and humans suggest that treatments to diminish, or bypass, the reduction in KGDHC might be beneficial in age-related neurodegenerative disorders...
  86. ncbi request reprint Genetically decreased spinal cord copper concentration prolongs life in a transgenic mouse model of amyotrophic lateral sclerosis
    Mahmoud Kiaei
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, New York 10021, USA
    J Neurosci 24:7945-50. 2004
    ..These findings provide evidence supporting a role for copper in the pathogenesis of FALS linked to SOD1 mutations...
  87. ncbi request reprint Matrix metalloproteinase-9 regulates TNF-alpha and FasL expression in neuronal, glial cells and its absence extends life in a transgenic mouse model of amyotrophic lateral sclerosis
    Mahmoud Kiaei
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 525 East 68th Street, New York, NY 10021, USA
    Exp Neurol 205:74-81. 2007
    ..This study provides new mechanism and suggests that MMP inhibitors may offer a new therapeutic strategy for ALS...
  88. ncbi request reprint High aggregate burden of somatic mtDNA point mutations in aging and Alzheimer's disease brain
    Michael T Lin
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY 10021, USA
    Hum Mol Genet 11:133-45. 2002
    ..Cytochrome oxidase activity correlated negatively with increasing mutational burden. These findings significantly bolster the mitochondrial theory of aging...
  89. ncbi request reprint Experimental therapeutics in transgenic mouse models of Huntington's disease
    M 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
  90. ncbi request reprint The energetics of Huntington's disease
    Susan E Browne
    Department of Neurology and Neuroscience, A 502, Weill Medical College of Cornell University, 525 East 68th Street, New York, New York 10021, USA
    Neurochem Res 29:531-46. 2004
    ..Here we discuss how animal models are redefining the role of energy metabolism in HD etiology...
  91. pmc Leucine-rich repeat kinase 2: a new player with a familiar theme for Parkinson's disease pathogenesis
    Chenjian Li
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY 10021, USA
    Proc Natl Acad Sci U S A 102:16535-6. 2005
  92. ncbi request reprint Striatal volume loss in HD as measured by MRI and the influence of CAG repeat
    H D Rosas
    Department of Neurology, Massachusetts General Hospital and Harvard Medical School 02114, USA
    Neurology 57:1025-8. 2001
    ..Progressive degeneration of the striatum is the pathologic hallmark of the disease. Little is known about the regional selectivity of the neurodegeneration and its relationship to the genetic expansion...
  93. ncbi request reprint Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2'dG
    S M Hersch
    Department of Neurology, MassGeneral Institute for Neurodegenerative Disease, Massachusetts General Hospital, Harvard Medical School, Charlestown, MA, USA
    Neurology 66:250-2. 2006
    ..Serum 8-hydroxy-2'-deoxyguanosine (8OH2'dG) levels, an indicator of oxidative injury to DNA, were markedly elevated in HD and reduced by creatine treatment...
  94. pmc Impaired brain creatine kinase activity in Huntington's disease
    S F Zhang
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY 10065, USA
    Neurodegener Dis 8:194-201. 2011
    ..Creatine kinase (CK) catalyzes ATP-dependent phosphorylation of creatine (Cr) into phosphocreatine (PCr), thereby serving as readily available high-capacity spatial and temporal ATP buffering...
  95. ncbi request reprint In vivo regulation of oxidative phosphorylation in cells harboring a stop-codon mutation in mitochondrial DNA-encoded cytochrome c oxidase subunit I
    F Pallotti
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 525 E 68th St, A-505, New York, NY 10021, USA
    J Biol Chem 276:46925-32. 2001
    ..Because of a small excess of COX capacity, the levels of expression of COX subunits exerted a relatively tight control on oxidative phosphorylation...
  96. ncbi request reprint N(epsilon)-(gamma-L-glutamyl)-L-lysine (GGEL) is increased in cerebrospinal fluid of patients with Huntington's disease
    T M Jeitner
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, USA
    J Neurochem 79:1109-12. 2001
    ..control CSF (228 +/- 36, n = 27); p < 0.0001. These data support the hypothesis that transglutaminase activity is increased in HD brain in vivo...
  97. ncbi request reprint Tissue inhibitors of matrix metalloproteinases are elevated in cerebrospinal fluid of neurodegenerative diseases
    S Lorenzl
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 525 East 68th Street Room A 501, New York, NY 10021, USA
    J Neurol Sci 207:71-6. 2003
    ..These findings show that TIMPs are elevated in the CSF of patients with neurodegenerative diseases suggesting a potential role of these endogenous inhibitors of matrix metalloproteinases in neurodegenerative diseases...
  98. ncbi request reprint Metal chelator decreases Alzheimer beta-amyloid plaques
    G K Gouras
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY 10021, USA
    Neuron 30:641-2. 2001
    ..The metal chelator, clioquinol, is reported by Cherny et al. (2001) to reduce Abeta plaques, presumably by chelation of Abeta-associated zinc and copper. This and other recent Abeta-modulating treatment approaches are discussed...
  99. ncbi request reprint Effects of coenzyme Q10 in early Parkinson disease: evidence of slowing of the functional decline
    Clifford W Shults
    Department of Neurosciences, Mail Code 0662, University of California San Diego, 9500 Gilman Dr, La Jolla, CA 92093 0662, USA
    Arch Neurol 59:1541-50. 2002
    ..Parkinson disease (PD) is a degenerative neurological disorder for which no treatment has been shown to slow the progression...
  100. ncbi request reprint Therapeutic effects of cystamine in a murine model of Huntington's disease
    Alpaslan 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...
  101. ncbi request reprint Huntington's disease of the endocrine pancreas: insulin deficiency and diabetes mellitus due to impaired insulin gene expression
    Ole A Andreassen
    Neurochemistry Laboratory, Massachusetts General Hospital, Boston, Massachusetts 02114, USA
    Neurobiol Dis 11:410-24. 2002
    ....