M. Flint Beal, M.D.

M. Beal

M. Flint Beal, MD is an internationally recognized authority on neurodegenerative disorders. He is Iniversity Professor  at the Brain and ind Institute of Weill Cornell Medical College.   Dr. Beal received his medical degree from the University of Virginia in 1976 and did his internship and first year residency in Medicine at New York-Cornell before completing his residency in Neurology at The Massachusetts General Hospital. He joined the neurology faculty at Harvard in 1983. Dr. Beal was Professor of Neurology at the Harvard Medical School and Chief of the Neurochemistry laboratory at Massachusetts General Hospital before moving to Cornell. Dr. Beal's research has focused on the mechanism of neuronal degeneration in Alzheimer's Disease, Huntington's Disease, Parkinson's Disease and amyotrophic lateral sclerosis (ALS).

Dr. Beal is the author or co-author of more than 500 scientific articles and more than 125 books, book chapters and reviews. He serves on the editorial boards of seven journals, the Journal of Neurochemistry, the Annals of Neurology, Journal of Molecular Neuroscience, Experimental Neurology and Neurobiology of Disease. Dr. Beal's research has focused on the mechanism of neuronal degeneration in Alzheimer's Disease, Huntington's Disease, Parkinson's Disease and amyotrophic lateral sclerosis (ALS). He has also been working on the development of novel neurochemical assays for assessing oxidative damage for use in clinical trials of new therapies for these disorders, as well as metabolomics for diagnosis.

Dr. Beal is a member of the Alpha Omega Alpha Medical Honorary Society and received the Derek Denny-Brown Neurological Scholar Award of the American Neurological Association. He has served on the Council and as Vice President of the American Neurological Association and on the Science Advisory Committees of the Hereditary Disease Foundation, Huntington's Disease Society of America, Parkinson's Disease Study Group, Parkinson' Disease Foundation, Bachman-Strauss Foundation, The ALS Association, and the American Health Assistance Foundation. Dr. Beal is also a member of the Institute of Medicine of the Namtional Academy of Sciences.

Selected Recent Papers (downloadable in Adobe Acrobat format*):

Energetics in the pathogenesis of neurodegenerative diseases

Mitochondrial DNA mutations in complex I and tRNA genes in Parkinson's disease

Neuroprotective Effects of Creatine in a Transgenic Mouse Model of Huntington's Disease

Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases. Nature 2006;443:787-795

Therapeutic effects of coenzyme Q10 and reduced COQ10 in the 1-methyl-4-phenyl-1, 2, 3, 6-tetrahydropyridine model of Parkinsonism.  J Neurochem 2008; 104:1613-1621

Metabolomic profiling to develop blood biomarkers for Parkinson's disease. Brain. 2008;131:389-96.

*If you don't have Acrobat Reader 3.0 or higher, you can download it from Adobe's website.


Recent Publications

Sugama S., Yang L, Cho BP, DeGiorgio L, Lorenzl S, Albers D, Beal MF. Age-related microglial activation in 1-methyl-4-phenyl-1, 2, 3, 6-tetrahydropyridine (MPTP)-induced dopaminergic neurodegeneration in C57BL/6 mice. Brain Res 2003;964:288-294

Paul S, Bogdanov MB, Matson WR, Metakis L, Jacobs J, Beal MF. Urinary 8-hydroxy-2'-deoxyguanosine, a metabolite of oxidized DNA, is not elevated in HIV patients on combiantion antiretroviral therapy. Free Rad Res 2003, 37:499-502

Wu AS, Kiaei M, Aguirre N, Crow JP, Calingasan NY, Browne SE, Beal MF. Iron porphyrin treatment extends survival in a transgenic animal model of amyotrophic lateral sclerosis. J Neurochem 2003:85:142-150

Lorenzl S, Albers DS, Relkin N, Ngyuen T, Hilgenberg S, Chirichigno J, Cudkowicz ME, Beal MF. Increased plasma levels of matrix metalloproteinase 9 in patients with Alzheimer's Disease. Neurochem Int 2003,43:191-196

Dedeoglu A, Kubilus JK, Yang L, Ferrante KL, Hersch SM, Beal MF, Ferrante RJ. Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's Disease transgenic mice. J Neurochem 2003,85:1359-1367

Zhang L, Ulug AM, Zimmerman RD, Lin M, Rubin M, Beal MF. The diagnostic utility of FLAIR imaging in clinically verified amyotrophic lateral sclerosis. J Magn Reson Imaging 2003,17:521-527

Gajewski CD, Lin MT, Cudkowicz ME, Beal MF, Manfredi G. Mitochondrial DNA from platelets of sporadic ALS patients restores normal respiratory functions in p0 cells. Exp Neurol 2003; 179:229-235

Lorenzl S, Albers DS, LeWitt PA, Chirichigno JW, Hilgenberg SL, Hilgenberg SL, Cudkowicz ME, Beal MF. Tissue inhibitors of matrix metalloproteinases are elevated in cerebrospinal fluid of neurodegenerative diseases. J Neurol Sci 2003, 207:71-76

Beal MF. Bioenergetic approaches for neuroprotection in Parkinson's Disease. Ann Neurol 2003; 53:S39-S48

Kaur D, Yantiri F, Rajagopalan S, Kumar J, Mo JQ, Boonplueang R, Viswanath V, Jacobs R, Yang L, Beal MF, DiMonte D, Volitaskis I, Ellerby L, Cherny RA, Bush AI, Andersen JK. Genetic or pharmacological iron chelation prevents MPTP-induced neurotoxicity in vivo: A novel therapy for Parkinson's Disease. Neuron 2003,37:899-909

Yang L, Sugama S, Chirichigno JW, Gregorio J, Lorenzl S, Shin DH, Browne SE, Shimizu Y, Joh TH, Beal M.F., Albers DS. Minocycline enhances MPTP toxicity to dopaminergic neurons. J Neurosc Res 2003;74:278-285

Klivenyi P., Ferrante R.J., Gardian G., Browne S., Pierre-Etienne C, Beal M.F. Increased survival and neuroprotective effects of BN-82451 in a transgenic mouse model of Huntington's disease. J Neurochem 2003;86:267-272

Lorenzl S., De Pasquale G., Segal A.Z., Beal, M.F. Dysregulation of the levels of matrix metalloproteinases and tissue inhibitors of matrix metalloproteinases in the early phase of cerebral ischemia. Stroke 2003;34,e37-e38

Klivenyi P., Gardian G., Calingasan N.Y., Yang L., Beal M.F. 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. J Mol Neurosci 2003;21:191-198

Lin M. T. and Beal MF. The oxidative damage theory of aging. Clin Neurosci Res 2003; 2:305-315

Barberi T., Klivenyi P., Calingasan N.Y., Lee H., Kawamata H., Loonam K., Perrier A.L., Bruses J., Rubio M. E., Topf N., Tabar V., Harrison N. L., Beal M.F., Moore M. A., Studer L. Neural subtype specification of fertilization and nuclear transfer embryonic stem cells and application in parkinsonian mice. Nat Biotechnol 2003; 21:1200-1207

Diano S., Matthews R.T., Patrylo P., Yang L., Beal M.F., Barnstable C.J., Horvath T.L.. Uncoupling protein 2 prevents neuronal death including that occurring during seizures: a mechanism for preconditioning. Endocrinology 2003;144:5014-5021

Andreassen OA., Ferrante RJ., Aamo TO., Beal MF., Jorgensen HA. Oral dyskinesias and histopathological alterations in susbstantia nigra after long-term haloperidol treatment of old rats. Neurosci 2003;122:717-725

Beal, M.F. Commentary - Alpha-synuclein and mitochondria: A tangled skein. Exp Neurol, in press

Beal M.F., Shults C.W. Effects of coenzyme Q10 in Huntington's disease and early Parkinson's disease. Biofactors 2003;18:153-161

Simon D.K., Lin M.T., Zheng L., Liu G.J., Ahn C.H., Kim L.M., Mauck W.M., Twu F., Beal M.F., Johns D.R. Somatic mitochondrial DNA mutations in cortex and substantia nigra in aging and Parkinson's Disease. Neurobiol Aging 2004;25:71-81

Ulug A.M., Grunewald T., Lin M.T., Kamal A.K., Filippi C.G., Zimmerman R.D., Beal M.F. Diffusion tensor imaging in the diagnosis of primary lateral sclerosis. J Magn Reson Imaging 2004;19:34-39

Browne S.E., Roberts II L.J., Dennery P.A., Doctrow S.R., Beal M.F. Barlow C., Levine R.L.. Treatment with a catalytic antioxidant corrects the neurobehavioral defect in ataxia-telangiectasia mice. Free Rad Biol Med 2004, 36:938-942

Klivenyi P., Kiaei M., Gardian G., Calingasan N.Y., Beal M.F. Additive neuroprotective effects of creatine and cyclooxygenase 2 inhibitors in a transgenic mouse model of ALS. J Neurochem 2004;88:576-582

Klivenyi P., Starkov A.A., Calingasan N.Y., Gardian G., Browne S.E., Yang L., Bubber P., Gibson G.E., Patel M.S., Beal M.F. Mice deficient in dihydrolipoamide dehydrogenase show incresed vulnerability to MPTP, malonate and 3-nitropropionic acid neurotoxicity. J Neurochem 2004, in press

Lorenzl S., Albers D.S., Chirichigno J.W., Augood S.J., Beal M.F. Elevated levels of matrix metalloproteinases-9 and -1 and of tissue inhibitors of MMPs, TIMP-1 and TIMP-2 in postmortem brain tissue of progressive supranuclear palsy. J Neurol Sci 2004; 218:39-45

Lorenzl S., Calingasan N., Yang L., Albers D.S., Shugama S., Gregorio J., Krell H. W., Chirichigno J., Joh T., Beal M.F. Matrix metalloproteinase-9 is elevated in 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-induced Parkinsonism in mice. Neuromolecular Med 2004, in press

Brownell A-L., Chen Y.I., Yu M., Wang X., Dedeoglu A., Beal M.F., Jenkins .G. 3-Nitroproionic acid induced neurotoxicity - assessed by ultra high resolution PET with comparison to MRI and MRS. J Neurochem 2004, in press

Klivenyi P., Calingasan N.Y., Stavrovskay I.G., Kristal B.S., Yang L., Petri S., Wieringa B., Beal M.F. Neuroprotective mechanisms of creatine occur in the absence of mitochondrial creatine kinase. Neurobiol Dis 2004, in press

Starkov A.A., Fiskum G., Chinopoulos C., Lorenzo B.J., Browne S.E. Patel M.S., Beal M.F Mitochondrial alpha-ketoglutarate dehydrogenase complex generates reactive oxygen species. J Biol Cem 2004' submitted

Li F., Calingasan N.Y., Fangmin Y., Mauck W.M., Almeida C. G., Takahashi R. H., Carlson G. A., Beal M.F., Lin M.T., Gouras G.K. Increased beta-amyloid levels and plaque burden in brains of APP mutant MnSOD heterozygous knockout mice. J Neurochem 2004, in press


Jenkins BG, Andreassen OA, Dedeoglu A, Leavitt B, Hayden  M, Borchelt D, Ross CA, Ferrante RF, Beal MF.  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 Neurchem 2005;95:553-562



Son JH, Kawamata H, Yoo MS, Kim DJ, Lee YK, Kim S, Dawson TM, Zhang H, Sulzer D, Yang L, Beal MF, Degiorgio LA, Chun HS, Baker H, Peng C.  Neurotoxicity and behavioral deficits associated with Septin 5 accumulation in dopaminergic neurons.   J Neurochem 2005;94:1040-1053



Cantuti-Castelvetri I, Lin MT, Zheng K, Keller-McGandy CE, Betensky RA, Johns DR, Beal MF, Standaert DG, Simon DK.  Somatic mitochondrial DNA mutations in single neurons and glia.  Neurobiol Aging 2005;26:1343-1355



Ferrante KL, Shefner J, Zhang H, Betensky R, O’Brien M, Yu H,  Fantasia M, Taft J, Beal MF, Traynor B, Newhall K, Donofriio P, Caress J,  Ashburn C, Freiberg B, O’Neill C, Paladench C, Walker T, Pestronuk A, Abrams B, Florence J, Renna R, Schierbecker J, Malkus B, Cudkowicz M.  Tolerance of high-dose (3,000 mg/day) coenzyme Q10 in ALS.  Neurology 2005;65:1834-1836


Nathan C, Calingasan N, Nezezon J, Ding A, Lucia MS, La Perle K, Fuortes M, Lin M, Ehrt S, Kwon NS, Chen J, Vodovotz Y, Kipiani K, Beal MF.  Protection from Alzheimer’s-like disease in the mouse by genetic ablation of inducible nitric oxide synthase.  J Exp Med 2005;202:1163-1169



Kiaei M, Kipiani K, Petri S, Chen J, Calingasan NY., Beal MF.  Celastrol blocks neuronal cell death and extends life in transgenic mouse model of amyotrophic lateral sclerosis.  Neurodegenerative Dis 2005;2:246-254



Hersch SM, Gevorkian S, Marder K, Moskowitz C, Feigin A, Cox M, Como P, Zimmerman C, Lin M, Zhang L, Ulug A, Beal MF, Matson W, Bogdanov M, Ebbel E, Zaleta A, Kaneko Y, Jenkins B, Hevelone N, Zhang H, Yu H, Schoenfeld D, Ferrante R, Rosas HD.  Creatine is safe, tolerable, bioavailable in brain and reduces serum 8OH2dG.  Neurology 2006;66:250-252



Elson JL, Hernstadt C, Preston G, Thal L, Morris CM, Edwardson JA, Beal MF, Turnbull DM, Howell N.  Does the mitochondrial genome play a role in the etiology of  Alzheimer’s Disease:  Hum Genet 2006;12:1-14



Lorenzl S, Narr S, Angele B, Krell HW, Gregorio J, Kiaei M, Pfister HW, Beal MF.  The matrix metalloproteinases inhibitor Ro 26-2853 extends survival in transgenic ALS mice.  Exp Neurol 2006, 200:166-171



Kaiei M, Kipiani K, Petri S, Gardian G, Choi D-K, Chen J, Calingasan NY, Schafer P, Muller GW, Stewart C, Hensley K, Beal MF.  Thalidomide and lenalidomide attenuate TNF-a/FasL mediated motor neuron death in a transgenic mouse model of ALS.  J Neurosci 2006, 26:2467-2473



Damiano M, Starkov AA, Petri S, Kipiani K, Kiaei M, Mattiazzi M, Beal MF, Manfredi G.  Neural mitochondrial Ca capacity impairment precedes the onset of motor symptoms in G93A Cu/Zn-superoxide dismutase mutant mice.  J Neurochem 2006;96:1346-1391



Klivenyi P, Siwek D, Gardian G, Yang L, Starkov A, Cleren C, Ferrante RJ, Kowall NW, Abeliovich A, Beal MF.  Mice lacking alpha-synucleain are resitant to mitochondrial toxins.  Neurobiol Dis 2006;21:541-548



Petri S, Kiaei M, Kipiani K, Chen J, Calingasan NY, Crow JP, Beal MF.  Additive neuroprotective effects of a histone deacetylase inhibitor and a catalytic antioxidant in a transgenic mouse model of amyotrophic lateral sclerosis.  Neurobiol  Dis 2006;22:40-49



Petri S, Kiaei M, Damiano M, Hiller A, Calingasan NY, Manfredi G, Szeto H, Beal MF.  Cell-permeable peptide antioxidants as a novel therapeutic approach in a mouse model of amyotrophic lateral sclerosis.  J Neurochem, 2006;98:1141-1148



Yang Y, Gehrke S, Imai Y, Huang Z, Ouyang Y, Wang J-W, Yang L, Beal MF, Vogel H, Lu B.  Muscle and dopaminergic degeneration caused by inactivation of Drosophila Pten-induced kinase (Pink1) is suppressed by Parkin.   Proc Natl Acad Sci, 2006, 103:10793-10798



Kwong JQ, Beal MF, Manfredi G.  The role of mitochondria in inherited neurodegenerative diseases.  J Neurochem, 2006;97:1659-1675



Abeliovich A, Beal MF.  Parkinsonism genes: culprits and clues.  J Neurochem 2006 Nov;99 (4):1062-72.



Browne SE, Yang L, Dimauro JP, Fuller SW, Licata SC, Beal MF.  Bioenergetics abnormalities in discrete cerebral motor pathways presage spinal cord pathology in the G93A SOD1 mouse model of ALS.  Neurobiol Dis, 2006;22:599-610



The Huntington Study Group PHAROS Investigators.  At risk for Huntington’s Disease.  Arch Neurol, 2006;63:991-998




Saydoff J, Garcia RAG, Browne SE, Liu L, Sheng J, Brenneman D, Hu Z, Cardin S, Gonzalez A, von Borstel RW, Gregorio J, Burr H, Beal MF.  Oral uridine pro-drug PN401 is neuroprotective in the R6/2 and N171-82Q mouse models of Huntington’s Disease.  Neurobiol Dis 2006;24:455-465



Lin MT and  Beal MF.  Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases.  Nature 2006;443:787-795



Browne SE. and Beal, MF.  Oxidative damage in Huntington’s disease pathogenesis.  Antioxid Redox Signal  2006;8:2061-2073



Lin MT and Beal MF. Alzheimer’s APP mangles mitochondria. Nat Med 2006;12:1241-1243



McGill JK, and Beal, M.F.  PGC-1alpha, a new therapeutic target in Huntington’s Disease?  Cell 2006;127:465-468



Roy NS, Cleren C, Singh SK, Yang L, Beal MF, Goldman SA.  Functional engraftment of human ES cell-derived dopaminergic neurons enriched by coculture with telomerase-immortalized midbrain astorcytes.  Nat Med 2006; 12:1259-1268



Beal MF and Lang A.  The proteasomal inhibiton model of Parkinson’s Disease:  “Boon or Bust”? Annals of Neurology 2006;60:158-161




Petri S, Kiaei M, Wille E, Calingasan NY, Beal MF.  Loss of Fas ligand-function improves survival in G93A-trangenic ALS mice.  J Neurological Sciences 2006;251:44-49



Rodriguez MC, Macdonald JR, Mahoney DJ, Parise G, Beal MF.  Tarnopolsky MA.  Beneficial effects of creatine, CoQ(10), and lipoic acid in mitochondrial disorders.  Muscle Nerve 2007;35:235-42



Thomas B,  von Coelln R, Mandir AS, Trinkaus DB, Farah MJ, Lim K-L, Calingasan NY, Beal MF, Dawson VL.  MPTP and DSP-4 susceptibility of substantia nigra and locus coeruleus catecholaminergic neurons in mice is independent of parkin activity.  Neurobiol Dis 2007;26:312-322



Beal MF, Bossy-Wetzel E, Finkbeiner S, Fiskum G, Giasson B, Johnson C,

Khachaturian ZS, Lee VM-Y, Nicholls D, Reddy H, Reynolds I, Teplow DB, Thal LJ, Trojanowski JQ, Walsh DM, Wetzel R, Wexler NS, Young AB, Bain L.  Common threads in neurodegenerative disorders of aging.  Alzheimer’s Dementia:  J Alzheimer’s Association 2007;2:322-326



Watanabe S, Nagano S, Duce J, Kiaei M, Li Q-X, Tucker SM, Tiwari A, Brown RH, Beal MF, Hayward LJ, Culotta VC, Yoshihara S, Sakoda S, Bush AI.  Increased affinity for copper mediated by cysteine 111 in forms of mutant superoxide dismutase 1 linked to amyotrophic lateral sclerosis. Free Radical Biology and Medicine 2007; 42:1534-1542



Petri S, Calingasan NY, Alsaied OA, Wille E, Kiaei M, Friedman JE, Baranova O, Chavez JC, Beal MF.  The lipophilic metal chelators DP-109 and DP-460 are neuroprotective in a transgenic mouse model of amyotrophic lateral sclerosis.  J Neurochemistry 2007;102: 991-1000



Kiaei M, Kipiani K, Calingasan NY, Wille E, Chen J, Heissig B, Rafii S, Lorenzl S, Beal MF.  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.  Exp Neurol 2007;205:74-81



Kim YS, Choi DH, Block ML, Lorenzl S, Yang L, Kim YJ, Sugama S, Cho BP, Hwang O, Browne SE, Kim SY, Hong J-S, Beal MF, John TH.  A pivotal role of matrix metalloproteinase-3 activity in dopaminergic neuronal degeneration via microglial activation.  FASEB 2007; 21:179-187



Goldman SA, Roy NS, Beal MF, Cleren C.  Large stem cell grafts could lead to erroneous interpretations of behavioral results?  Nat Med 2007;205:74-81



Constantinescu R, McDermott MP, DiCenzo R, de Blieck EA, Hyson HC, Beal MF, Bednarczyk EM, Bgdanov M, Metakis LJ, Browne SE, Lorenzo BJ, Ravina B, Kieburtz K.  A randomized study of the bioavailability of different formulations of coenzyme Q10 (ubiquinone). J Clin Pharmacol 2007;47:1580-1586



Stack EC, Dedeoglu A, Smith KM, Cormier K, Kubilus JK, Bogdanov M, Matson WR, Yang L, Jenkins BG, Luthi-Carter R, Kowall NW, Hersch SM, Beal MF, Ferrante RJ.  Neuroprotective effects of synaptic modulation in Huntington’s Disease R6/2 mice.  J Neurosci 2007;27:12908-12915



Strand AD, Baquet ZC, Aragaki AK, Holmans P, Yang L, Cleren C, Beal MF, Jones L, Kooperberg C, Olson JM, Jones KR.  Expression profiling of Huntington’s disease models suggests that brain-derived neurotrophic factor depletion plays a major role in striatal degeneration.  J Neurosci 2007;27:11758-11768



Thomas B and Beal MF.  Parkinson’s disease.  Hum Mol Genet 2007;16:183-94



Cleren C, Yang L, Lorenzo B, Calingasan NY, Schomer A, Sireci A, Wille EJ, Beal MF.  Therapeutic effects of coenzyme Q10 and reduced COQ10 in the 1-methyl-4-phenyl-1, 2, 3, 6-tetrahydropyridine model of Parkinsonism.  J Neurochem 2008; 104:1613-1621



Reddy H, and Beal MF.  Amyloid beta, mitochondrial dysfunctionand synaptic damage: implications for cognitive decline in aging and Alzheimer’s Disease.  Trends in Molecular Medicine, 2008;14:45-53



Langley B, D’Annibale MA, Suh K., Ayoub I, Tolhurst A, Basan B., Yang L, Ko B, Fisher M, Cho S, Beal MF, Ratan Rr.  Pulse inhibition of histone deacetylases induces complete resistance to oxidative death in cortical neurons without toxicity and reveals a role for cytoplasmic p21 waf/cip1 in cell cycle-independent neuroprotection.  J Neurosci 2008;28:163-176



Bogdanov M, Matson WR, Wang L, Matson T, Saunders-Pullman R, Bressman SS, Beal MF. Metabolomic profiling to develop blood biomarkers for Parkinson's disease. Brain. 2008;131:389-96.



Lorenzl S, Buerger K, Hampel H, Beal MF. Profiles of matrix metalloproteinases and their inhibitors in plasma of patients with dementia.  International Psychogeriatrics 2008;20:67-76



Chaturvedi RK and Beal MF.  Mitochondrial approaches for neuroprotection.  Ann New York Acad Sci 2008;1147:395-412



Adhihetty P and Beal MF.  Creatine and its potential therapeutic value for targeting cellular energy impairment in neurodegenerative diseases.   NeuroMol Med 2008, 10:275-290



Akopian G, Crawford C, Beal, MF, Cappelletti M, Jakowec MW, Petzinger GM, Zheng L, Gheorghe SL, Reichel CM, Chow R, Walsh JP.  Decreased striatal dopamine release underlies increased expression of long-term synaptic potentiation at corticostriatal synapses 24 h after 3-nitropropionic-acid-induced chemical hypoxia.  J Neurosci 2008; 28:9585-9597



Mitsumoto H, Santella RM, Liu X, Bogdanov M, Zipprich J, Wu HC, Mahata J, Kilty M, Bednarz K, Bell D, Gordon PH, Hornig M, Mehrazin M, Naini A, Beal MF, Factor-Litvak P.  Oxidative stress biomarkers in sporadic ALS.  Amyotrophic Lateral Scler 2008;9:177-83



Choi DH, Kim EM, Son HJ, Joh TH, Kim YS, Kim D, Beal MF, Hwang O.  A novel intracellular role of matrix metalloproteinase-3 during apoptosis of dopaminergic cells.  J Neurochem 2008;106:405-15



Banerjee R, Starkov AA, Beal MF, Thomas B.  Mitochondrial dysfunction in the limelight of Parkinson’s disease pathogenesis.   BBA Mol Basis of Dis 2009;1792:651-663



Henchcliffe C and Beal MF.  Mitochondrial biology and oxidative stress in Parkinson disease pathogenesis.  Nat Clin Prac Neurol. 2008;4:600-609



Chaturvedi RK and Beal MF.  PPAR: A therapeutic target in Parkinson’s disease J. Neurochem 2008;106:506-18



Johansen KK, Wang L, Aasly JO, White LR, Matson WR,  Henchcliffe C, Beal MF, Bogdanov M.    Metabolic profiling in LRRK2-related Parkinson’s Disease. Annals of Neurology 2008, in press



Karuppagounder SS, Pinto JT, Xu H, Chen LH, Beal MF, Gibson GE.   Dietary supplementation with resevatrol reduces plaque pathology in a transgenic model of Alzheimer’s Disease.  Neurochem Intl 2008, 54:111-118



Yang Y, Ouyang Y, Yang L, Beal MF, McQuibban A, Vogel lH, Lu B.  PINK1 regulates mitochondrial dynamics through interaction with the fission/fusion machinery.  Proc Natl Acad Sci USA 2008;105:7070-7075



Beal, M.F.  The urokinase system of plasminogen activator plays a role in amyotrophic lateral sclerosis (ALS) pathogenesis.  Exp Neurol 2008;211:332-333



Karuppagounder SS, Xu H, Shi Q, Chen LH, Pedrini S, Pechman D, Baker H, Beal MF, Gandy SE, Gibson GE.  Thiamine deficiency induces oxidative stress and exacerbates the plaque pathology in Alzheimer’s mouse model. Neurobiol Aging 2009 Apr 9 [Epub ahead of print]; 30:1587-1600 



Henchcliffe C, Shungu DC, Mao X, Huang C, Nirenberg MJ, Jenkins BG, Beal MF. Multinuclear magnetic resonance spectroscopy for in vivo assessment of mitochondrial dysfunction in Parkinson’s Disease.  Ann NY Acad Sci 2008:1147:206-220



Yang L, Calingasan NY, Lorenzo BJ.  Attenuation of MPTP neurotoxicity by rolipram, a specific inhibitor of phosphodiesterase IV.  Exp. Neurol 2008, 211;311-4



Starkov AA and Beal MF.  Portal to Alzheimer’s disease.  Nat Med 2008;14:1020-1021



Calingasan NY, Ho DJ, Wille EJ, Campagna MV, Ruan J, Dumont M, Yang L, Shi Q, Gibson GE, Beal MF.   Influence of mitochondrial enzyme deficiency on adult neurogenesis in mouse models of neurodegenerative diseases.  Neuroscience  2008;153:986-996



Dumont M, Wille E., Stack C,  Calingasan NY, Beal MF, Lin MT.  Reduction of oxidative stress, amyloid deposition and memory deficit by manganese superoxide dismutase overexpression in a transgenic mouse model of Alzheimer’s disease. FASEB J 2009;23:2459-2466



Liu W, Vives-Bauza C., Acin-Perez R, Yamamoto A, Tan Y, Li Y, Magrane J, Stavarache MA, Shaffer S, Chang S, Kaplitt MG, Huang XY, Beal MF, Manfredi G, Li C.  PINK1 defect causes mitochondrial dysfunction, proteasomal deficit and alpha-synuclein aggregation in cell culture models of Parkinson’s disease. PLoS ONE 2009;4:e4597



Dumont M, Wille E, Calingasan NY, Tampellini D, Williams C, Gouras GK, Liby K, Sporn M, Beal MF, Lin MT.  Triterpenoid CDDO-methylamide improves memory and decreases amyloid plaques in a transgenic mouse model of Alzheimer’s disease.  J Neurochem 2009;109:502-512



Yang L, Calingasan NY, Wille EJ, Cormier K, Smith K, Ferrante RJ, Beal MF.  Combination therapy with Coenzyme Q10 and creatine produces additive neuroprotective effects in models of Parkinson’s and Huntington’s Diseases.  J Neurochem 2009;109:1427-1439



Yang L, Zhao K, Calingasan NY, Luo G, Szeto HH, Beal MF.  Mitochondria targeted peptides protect against 1-methyl-4-phenyl-1, 2, 3, 6-tetrahydropyridine neurotoxicity.  Antioxid Redox Signal 2009;11:2095-2104



Li Y, Liu W, Oo TF, Wang L, Tang Y, Jackson-Lewis V, Zhou C, Geghman K, Bogdanov M, Przedborski S, Beal MF, Burke RE, Li C.  Mutant LRRK2 (R1441G) BAC transgenic mice recapitulate cardinal features of Parkinson’s disease.  Nat Neurosci 2009;12:826-828




Chaturvedi RK, Adhihetty P, Shukla S, Hennessy T, Calingasan N, Yang L, Starkoiv A, Kiaei M, Canella M, Sassone J, Ciammola A, Squitieri F, Beal MF. Impaired PGC-1alpha function in muscle in Huntington’s disease.  Hum Mol Genet 2009;18:3048-3065



Yang L, Calingasan NY, Thomas B, Chaturvedi RK, Kiaei M, Wille EJ, Liby KT, Williams C, Royce D, Risingsong R, Musiek ES, Morrow JD, Sporn M, Beal MF.  Neuroprotective effects of the triterpenoid, CDDO methyl amide, a potent inducer of Nrf2-mediated transcription.  PLoS One 2009; 4:e5757



Cristovao AC, Choi D, Baltazar G, Beal MF, Kim YS. The role of NADPH oxidase 1-derived reactive oxygen species in paraquat-mediated dopaminergic cell death.  Antioxid Redox Signal 2009;11;2105-2118



Dumont M, Ho DJ, Calingasan NY, Xu H, Gibson G, Beal MF.  Mitochondrial dihdrolipoyl succinyltransferase deficiency accelerates amyloid pathology and memory deficit in a transgenic mouse model of amyloid deposition.  Free Radic Biol Med 2009;47:1019-1027



Daher JPL, Ying M, Banerjee R, McDonald RS, Hahn MD, Yang L, Beal MF, Thomas B, Dawson VL, Dawson TM, Moore DJ.  Conditional transgenic mice expressing C-terminally truncated human alpha-synuclein (aSyn119) exhibit reduced striatal dopamine without loss of nigrostriatal pathway dopaminergic neurons.   Mol Neurodegen 2009;4:34



Lee DW, Rajagopalan, Siddiqui A, Gwiazda R, Yanl L, Beal MF, Ratan RR and Andersen JK.  Inhibition of prolyl hydroxylase protects against MPTP-induced neurotoxicity: model for the potentialinvolvement of the hypoxia-inducible factor pathway in Parkinson’s Disease.   J Biol Chem 2009;284:29065-29076



Yang L, Shi Q, Ho DJ, Starkov AA, Wille EJ, XU H, Chen HL, Zhang S, Stack CM, Calingasan NY, Gibson GE, Beal MF.  Mice deficient in dihydrolipoyl succinyltransferase show increased vulnerability to mitochondrial toxins.  Neurobiol Dis 2009;36:320-330



Marder K, Zhao H, Eberly S, Tanner CM, Oakes D, Shoulson I, Huntington Study Group.  Dietary intake in adults at risk for Huntington disease:analysis of PHAROS research participants.  Neurology 2009;4:385-392



Ascherio A, LeWitt PA, Xu K, Eberly S, Watts A, Matson WR, Marras C, Kieburtz K, Rudolph, A, Bogdanov MB, Schwid SR, Tennis M, Tanner CM, Beal MF, Lang AE, Oakes D, Fahn S, Shoulson I,  Schwarzschild MA on behalf of the PSG DATATOP investigators. Urate as a predictor of the rate of clinical decline in Parkinson disease. Arch. Neurol. 2009,66:1460-1468



Faust K, Gehrke S, Yang Y, Yang L, Beal MF, Lu B.  Neuroprotective effects of compounds with antioxidant and anti-inflammatory properties in a Drosophila model of Parkinson’s disease.  BMC Neurosci 2009;10:109



Neymotin A, Petri S, Calingasan NY, Wille E, Schafer P, Stewart C, Hensley K, Beal MF, Kiaei M.  Lenalidomide (Revlimid) Administration at symptom onset is neuroprotective in a mouse model of amyotrophic lateral sclerosis.  Exp Neurol 2009; 220:191-197



Johansen KK, Wang L, Aasly JO, White LR, Matson WR, Henchcliffe C, Beal MF, Bogdanov M.  Metabolomic profiling in LRRK2-related Parkinson’s Disease.  PLoS One 2009;4:e7551



Andres-Mateos E., Mejias R., Sasaki M., Li X., Lin BM., Biskup S., Zhang L., Banerjee R., Thomas B., Yang L., Liu g., Beal MF., Huso DL, Dawson TM, Dawson VL.  Unexpected lack of hypersensitivity in LRRK2 knock-out mice to MPTP(1methyl-4-Phenyl-1,2,3,6-Tetrahydropyridine).  J Neurosci 2009;29:15846-15850



Spindler M, Beal MF, Henchcliffe C.  Coenzyme Q10 effects in neurodegenerative disease.  Neuropsychiatr Dis Treat 2009;5:597-610



Beal MF.  Therapeutic approaches to mitochondrial dysfunction in Parkinson’s Disease.  Parkinsonism Relat Disord 2009; Suppl 3:S189-94



Daher JP, Ying M, Banerjee R, McDonald RS, Hahn MD, Yang L, Beal MF, Thomas B, Dawson VL, Dawson TM, Moore DJ.  Conditional transgenic mice expressing C-terminally truncated human alpha-synuclein (alphaSyn119) exhibit reduced striatal dopamine without loss of nigrostriatal pathway dopmainergic neurons.  Mol Neurodegener 2009;4:34



Gibson GE, Starkov A, Blass JP, Ratan RR, Beal MF.  Cause and consequence: Mitochondrial dysfunction initiates and propagates neuronal dysfunction, neuronal death and behavioral abnormalities in age-associated neurodegenerative diseases.  Biochim Biophys Acta 2010;1802:122-134



Cleren C, Calingasan NY, Starkov A, Jacquard C, Chen J, Brouillet E, Beal MF.  Promethazine protects against 3-nitropropionic acid-induced neurotoxicity.  Neurochem Intl 2010, 56:208-212



Yin F., Banerjee R., Thomas B., Zhou P., Qian L., Jia T., Ma X., Ma Y., Iadecola C., Beal MF., Nathan CM.,  Ding A.  Exaggerated inflammation, impaired host defense and neuropathology in progranulin-deficient mice.  J Exp Med 2010; 207:117-128



Dumont M., Wille E., Calingasan NY., Nathan C., Beal FM., Lin MT.  N-Iminoethyl-L-Lysine improves memory and reduces amyloid pathology in a transgenic mouse model of amyloid deposition.  Neurochem Int 2010;56:345-351.



Ho DJ, Calingasan NY, Wille E, Dumont M., Beal MF.  Resveratrol protects against peripheral deficits in a mouse model of Huntington’s Disease.  Exp Neurol 2010; 225:74-84



Chaturvedi RK, Calingasan NY, Yang L, Hennessey T, Johri A, Beal MF.  Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington’s disease following chronic energy deprivation. Hum Mol Genet 2010;19:3190-3205



Dumont M, Lin MT, Beal MF.  Mitochondria and antioxidant targeted therapeutic strategies for Alzheimer’s disease.  J Alzheimers Dis 2010:20 Suppl 2:S633-643



Stack C, Ho D, Wille E, Calingasan NY, Williams C, Liby K, Sporn M, Dumont M, Beal MF. Triterpenoids CDDO-ethyl amide and CDDO-trifluoroethyl amide improve the behavioral phenotype and brain pathology in a transgenic mouse model of Huntington’s disease.  Free Radic Biol Med 2010; 49:147-58, PMID:20338236  



Thomas B, Beal MF.  Mitochondrial therapies for Parkinson’s Disease.  Mov Disord 2010:25 Suppl 1:S155-60



McConoughey SJ, Nietskaya Z, Karpisheva K, Langley BC, Cooper AJL, Beal MF, Coppola G, Geschwind D, Ryu H, Li X, Graham R, Marsh LJ, Thompson L, Ratan RR.  Transglutaminase 2 inhibition ameliorates transcriptional dysregulation in models of Huntington’s disease.  EMBO Mol Med 2010; 2:349-370



Kim J, Moody J, Edgerly C, Bordiuk O, Cormier K, Smith K, Beal MF, Ferrante R.  Mitochondrial loss, dysfunction, and altered dynamics in Huntington’s Disease.  Hum Mol Genet 2010; 19:3919-3935



The Huntington Study Group Pre2CARE Investigators, Hyson HC, Kieburtz K, Shoulson I, McDermott M, Ravina B, deBlieck EA, Cudkowicz ME, Ferrante RJ, Como P, Frank S, Zimmerman C, Cudkowicz ME, Ferrante K, Newhall K, Jennings D,  Kelsey T, Walker F, Hunt V, Daigneault S, Goldstein M, Weber J, Watts A, Beal MF, Browne SE, Metakis LJ.  Safety and tolerability of high-dosage coenzyme Q10 in Huntington’s Disease and healthy subjects.  Movement Disorders; 25:1924-1928



Beal MF.  Parkinson’s Disease:  A Model Dilemma.  Nature, 2010, 466;S8-S10



Johri A, Beal MF.  Hunting-ton for new proteases: MMPs as the new target?  Neuron 2010, 67:171-173



Yin F, Dumont M, Banerjee R, Ma Y, Li H, Lin MT, Beal MF, Nathan C, Thomas B, Ding A. Behavioral deficits and progressive neuropathology in progranulin-deficient mice: a mouse model of frontotemporal dementia.  FASEB J 2010,24: 4639-4647 PMID:  20667979



Banerjee R, Beal MF, Thomas B.  Autophagy in Neurodegeneration: Pathogenic roles and therapeutic implications.  Trends in Neurosciences 2010, 33:541-549



Choi DH, Hwang O, Lee KH, Lee J, Beal F, Kim YS.  DJ-1 clevage by matrix metalloproteinase 3 mediates oxidative stress induced dopaminergic cell death.  Antioxid Redox Signal 2010;14:2137-2150



Yao J, Hennessey T, Flynt A, Lai E, Beal M F, Lin MT.  MicroRNA-related cofilin abnormality in Alzheimer’s disease.  PLoS1 2010, 5:e15546



Dumont  M, Beal MF.  Neuroprotective strategies involving ROS in Alzheimer’s disease.  Free Rad Biol Med 2011;51:1014-1026.



Beal MF.  Neuroprotective effects of creatine.  Amino Acids 2011, 40:1305-1313



Zhang SF, Hennessey T, Starkova NN, Beal MF, Starkov AA.  Impaired brain creatine kinase activity in Huntington’s Disease. Neurodegenerative Dis  2010 doi:10.1159/000321681



Crawford CA, Akopian G, Ring J, Jakowec MW, Petzinger GM, Andersen JK, Vittozzi-Wong P, Wang K, Farley CM, Charntikov S, Mitroi D, Beal MF, Chow R, Walsh JP.  Acute and long-term response of dopamine nigrostriatal synapses to a single low dose episode of 3-nitroprionic acid-mediated chemical hypoxia.  Synapse 2011, 65:339-350 PMID: 20730800



Dumont M, Stack C, Elipenhali C, Calingasan NY, Wille E, Beal MF.  Apocynin administration doe not improve behavioral and neuropathological deficits in a transgenic mouse model of Alzheimer’s disease.  Neurosci Letts 2011, 492:150-154



Choi DH, Kim YJ, Kim YG, Joh TH, Beal MF, Kim YS.  The role of matrix metalloproteinase 3-mediated alpha-syuclein cleavage in dopaminergic cell death.  J Biol Chem 2011, 286:14168-14177. PMID:21330369



Neymotin A, Calingasan NY, Wille EJ, Naseri NN,  Petri S, Damiano M, Liby KT, Risingsong R, Sporn MB, Beal MF, Kiaei M.  Neuroprotective effect of Nrf2/ARE activators, CDDO- trifluoroethylamide in a mouse model of amyotrophic lateral sclerosis.  Free Rad Biol Med, 2011, 51:88-96  PMID: 21457778



Ramonet D, Daher JPL, Lin BM, Stafa K, Kim J, Banerjee R, Westerlund M, Pletnikova O, Glauser L, Yang L, Liu Y, Swing DA, Beal MF, Tronscoso JC, McCaffery M, Jenkins NA, Copeland NG, Galter D, Thomas B, Lee MK, Dawson TM, Dawson VL, Moore DJ.  Dopaminergic neuronal loss, reduced neurite complexity and autophagic abnormalities in transgenic mice expressing G2019S mutant LRRK2.  PLoS One 2011; 6:e18568. PMID 21494637.



Johri A, Chaturvedi RK, Beal MF.  Hugging tight in Huntington’s.  Nat Med 2011;17:514, PMID: 21457778



Thomas B, Banerjee R, Starkova N, Zhang S, Calingasan N, Yang L, Wille E, Lorenzo B, Ho D, Beal F, Starkov A.  Mitochondrial permeability transition pore component cyclophilin D distinguishes nigrostriatal dopaminergic death paradigms in the MPTP-mouse model of Parkinson’s disease.  Antioxid & Redox Signal 2012, 16:855-868 PMID: 21529244



Johri A, Starkov A, Hennessey T, Sharma A, Yang L, Beal MF.  Truncated peroxisome  proliferator activated receptor – Coactivator 1 (PGC1) splice variant is severely altered in Huntington’s Disease.  Neurodegen Dis 2011,8:496-503.PMID:21757867




Dumont D, Kipiani K, Yu F, Wille E, Katz M, Calingasan NY, Gouras GK, Lin MT, Beal MF. 

Coenzyme Q10 decreases amyloid pathology and improves behavior in a transgenic mouse model of Alzheimer’s disease.  J Alzheimer’s Dis 2011; 27:211-223.  PMID:21799249



Dumont M, Stack C, Elipenahli C, Jainuddin S, Gerges M, Starkova N, Yang L, Starkov A, Beal F.  Behavioral deficit, oxidative stress and mitochondrial dysfunction precede tau pathology in P301S transgenic mice.  FASEB J 2011, 25:4063-4072 PMID 21825035



Neymotin, A., Calingasan, N.Y., Wille, E., Naseri, N., Petri, S., Damiano, M., Liby, K.T., Risingsong, R., Sporn, M., Beal, M.F., Kiaei, M. Neuroportective effect of Nrf2/ARE activators, CDDO ethylamide and CDDO trifluoroethylamide, in a mouse model of amyotrophic lateral sclerosis.  Free Radic Biol Med 2011,51:88-96 PMID 21457778



Johri A., Calingasan NY, Hennessey TM, Sharma A, Yang L., Wille E, Chandra A, Beal MF.  Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington’s disease.  Hum Mol Genet, 2012, 21:1124-1137  PMID 22095692



Henchcliffe, C., Dodel R., Beal, M.F.  Biomarkers of Parkinson’s disease and dementia with Lewy bodies.  Prog Neurobiol 2011, 95:601-613. PMID: 21983334



Kriks, S., Shim, J-W., Piao, J., Ganat, Y.M., Wakeman, D.R., Xie, Z., Carillo, L., Auyeung, G., Buch, A., Yang, L., Beal, M.F., Surmeier, J., Kordower, J.H., Tabar, V., Studer, L.  Derivatation of authentic midbrain dopamine neurons from human ESCs results in highly efficient engraftment in murine and primate models of PD.  Nature, 2011, 480:547-551. PMID: 22056989



Johri, A and Beal, MF. Antioxidants in Huntington’s disease.  Biochim Biophys Acta Molecular Basis of Disease 2012,1822: 664-674. PMID:22138129



Manji, H., Kato, T., Di Propsero, N.A., Ness, S., Beal, M.F., Krams, M., Chen, G.  Mitochondrially mediated plasticity in major psychiatric disorders.  Nat Rev Neurosci 2012, 13:293-307.  PMID: 22510887



Yao, J., Lin, M., Beal, M.F. Mitochondrial dysfunction in neurodegenerative diseases.  Neurodegenerative Diseases.  Nature Neurosci Rev 2012, in press



Choi DH, Cristovao AC, Guhathakurta S, Lee J, Toh TH, Beal MF, Kim YS. NADPH oxidase 1-mediated oxidative stress leads to dopamine neuron death in Parkinson’s disease.  Antioxid Redox Signal  2012,16:1033-1045. PMID:22098189



Yerramilli-Rao P, Beal MF, Watanabe D, Kieburtz K, Blieck EA, Kitano M, Hosoe K, Funahasi I, Cudkowicz ME.  Oral repreated-dose toxicity studies of coenzyme Q in beagle dogs.  Int J Toxicol 2012,31:58-69. PMID: 22267890



Islam R, Yang L, Sah M, Kannan K, Anamani D, Vijayan C, Kwok J, Cantino ME, Beal MF, Fridell YW.  A neuroprotective role of the human uncoupling protein 2 (hUCP2) in a Drosophila Parkinson’s Disease model. Neurobiol Dis 2012, 46:137-146. PMID: 22266335



Elipenahli, C., Stack, C., Jainuddin, S., Gerges, M., Yang, L., Starkov, A., Beal, M.F., Dumont, M.  Behavioral improvement after chronic administration of Coenzyme Q10 in P301S transgenic mice.  J Alzheimers Dis 2012, 28:173-182, PMID:21971408



Lin MT, Cantuti-Castelvetri I, Zheng K, Jackson KE, Tan YB, Arzberger T, Lees AJ, Betensky RA, Beal MF, Simon DK. Somatic mitochondrial DNA mutations in early Parkinson and incidental Lewy Body disease.  Ann Neurol 2012, 71:850-854. PMID 22718549



Gollamudi S, Johri A, Calingasan NY, Yang L, Elemento O, Beal MF. Concordant signaling

 pathways produced by pesticide exposure in mice correspond to human Parkinson’s

Disease pathways.  PLoS One, 2012; 7:e36191.  PMID: 22563483.



Johri A and Beal MF.  Muscling in on PGC-1alpha for improved quality of life in ALS.  Cell

Metabolism 2012, 15:567-569.  PMID: 22560208.



Chaturvedi RK, Hennessey T, Johri A, Tiwari S, Mishra D, Agarwal S, Kim YS, Beal MF.

Transducer of regulated CREB-binding proteins (TORCs) transcription and function is

 impaired in Huntington’s disease. Hum Mol Gen, 2012, 21:3474-3488.  PMID: 22589249.




Johri A, Beal MF. Mitochondrial dysfunction in neurodegenerative diseases. J Pharmacol

 Exp Ther, 2012, 342: 619-630 PMID: 22700435.



Kaidery NA, Banerjee R, Yang L, Smirnova NA, Hushpulian DM, Liby KT, Williams CR,

Yamamoto M, Kenslet TW, Ratan RR, Sporn MB, Beal MF, Gazaryan IG, Thomas B.

Targeting Nrf2-mediated gene transcription by extremely potent synthetic triterpenoids

attenuate dopaminergic neurotoxicity in the MPTP-mouse model of Parkinson’s disease.

 Antioxid Redox Signal 2013; 18:139-157 PMID: 22746536.



Dumont M, Stack, C, Elipenahli C, Jainuddin S, Gerges M, Starkova N, Calingsan NY,

Yang L, Tampellini D, Starkov A, Chan RB, Pujol A, Beal MF.  Bezafibrate administration

improves behavioral deficits and tau pathology in P301S mice.  Human Mol Gen, 2012, 21:

5091-5105 PMID: 22922230.



Yao J, Ho D, Calingasan N, Pipalia NH, Lin M, Beal MF.  Neuroprotection by cyclodextrin

in cell and mouse models of Alzheimer’s disease. J Exp Med 2012, 209, 2501-2513 PMID:




Chaturvedi R, Beal MF  Mitochondria targeted therapeutic approaches in Parkinson’s and

Huntington’s diseases.  Mol Cell Neurosci 2013, 55:101-114. PMID: 23220289



Elm JJ. NINDS NET-PD Investigators.  Design Innovations and baseline findings in a long-

term Parkinson’s trial:  the National Institute of Neurological Disorders and Stroke

Exploratory Trials in Parkinson’s Disease Long-Term Study-1.  Mov Disord. 2012, 27:

1513-1521. PMID: 23079770



Ravina B, Marek K, Eberly S, Oakes D, Kurlan R, Ascherio A, Beal MF, Beck J, Flagg E,

Galpern WR, Harman J, Lang AE, Schwarzchild M, Tanner C, Shoulson I.  Dopamine

transporter imaging is associated with long-term outcomes in Parkinson’s disease.  Mov

Disord. 2012, 27: 1392-1397. PMID: 22976926



Franco MC, Ye Y, Refakis C, Feldman J, Stokes A, Basso M, Fernandez de Mera R,

Sparrow N, Calingasan N, Kiaei M, Rhoads T, Ma T, Grumet M, Barnes S, Beal MF,

Beckman J, Mehl R, Estevez A. Nitration of Hsp90 Induces Cell Death.  Proc Natl Acad Sci

 2013, 110:1102-1111. PMID: 23487751



Kiss G, Konrad C, Doczi J, Starkov A, Kawamata H, Manfredi G, Zhang S, Gibson G, Beal

MF, Adam-Vizi, V, Chinopoulos C. The negative impact of alpha-ketoglutarate

dehydrogenase complex deficiency on matrix substrate-level phosphorylation.  FASEB

2013, 27:2392-2406  PMID:23475850



Chaturvedi RK, Beal MF.  Mitochondrial diseases of the brain.  Free Radic Biol Med 2013,

63:1-29. PMID:23567191



Morato L, Galino J, Ruiz M, Calingasan NY,  Starkova A, Dumont M, Naudi A, Martinez JJ,

Aubourg P, Portero-Otin M, Pamplona R, Galea E, Beal MF, Ferrer I, Pujol A.  Pioglitazone

halts axonal degeneration in a mouse modle of X-linked adrenoleukodystrophy. Brain

2013, 136:2432-2443. PMID:23794606



Damiano M, Diguet E, Malgorn C, Galvan L, Petit F, Benhaim L, Guillermier M, Houitte D,

Dufour N, Hantraye P, Canals JM, Alberch J, Delzescaux T, Deglon N, Beal MF, Brouillet

E. A role of mitochondrial complex II defects in genetic models of Huntington’s disease

 Expressing N-terminal fragments of mutant huntingtin.  Human Mol Gen 2013, 22:3869-

3882. PMID:23720495.



Inoue K, Rispoli J, Yang L, Macleod D, Beal MF, Klann E, Abeliovich A.  Coordinate

Regulation of Mature Dopaminergic Axon Morphology by Macroautophagy and the PTEN

Signaling Pathway. PLoS Genet 2013, 9:e1003845. PMID 24098148



Johri A, Chandra A, Beal MF.  PGC-1alpha, mitochondrial dysfunction and Huntington’s

disease.  Free Radic Biol Med 2013; 62:37-46. PMID:23602910.



The Parkinson Study Group SURE-PD Investigators. Schwartzchild MA, Ascherio A, Beal MF, Cuddkowicz ME, Curhan GC, Hare JM, Hooper DC, Kieburtz KD, Macklin EA, Oakes D, Rudolph A, Shoulson I, Tennis MK, Espay AJ, Gartner M, Hung A, Bwala G, Lenehan R, Encarnacion E, Ainslie M, Castillo R, Togasaki D, Barles G, Friedman JH, Niles L, Carter JH, Murray M, Goetz CG, Jaglin J, Ahmed A, Russell DS, Cotto C, Goudreau JL, Russell D, Parashos SA, Ede P, Saint-Hilaire MH, Thomas CA, James R, Stacy MA, Johnson J, Gauger L, Antonelle de Marcaida J, Thurlow S, Isaacson SH, Carvajal l, Rao J, Cook M, Hope-Porche C, McClurg L, Grasso DL, Logan R, Orme C, Ross T,  Inosine to Increase Serum and Cerebrospinal Fluid Urate in Parkinson Disease:  A Randomized Clinical Trial.  JAMA Neurol 2014 71:141-150.PMID 24366103.



Weiduschat N, Mao X, Beal MF, Nirenberg M, Shungu D. Henchcliffe C. Sex differences

 in cerebal energy metabolism in Parkinson’s disease:  a phosphorus magnetic resonance

spectroscopic imaging study.  Parkinsonism and Related Disorders 2014. Epub PMID




The Parkinson Study Group QE Investigators, Beal MF, Oakes D, Shoulson I, Henchcliffe

 C et al.  A Randomized Clinical Trial of High Dosage Coenzyme Q10 in Early Parkinson

 Disease:  No Evidence of Benefit. JAMA Neurology 2014 published on line PMID:




Chandra A, Johri A, Beal MF.  Prospects for neuroprotective therapies in prodromal Huntington’s disease.  Mov Disord 2014, 3:285-93. PMID: 24573776



Stack C, Jainuddin S, Elipenahli C, Gerges M, Starkova N, Starkov AA, Jove M, Portero-Otin M, Launay N, Pujol A, Kaidery NA, Thomas B, Tampellini D, Beal MF, Dumont M.  Methylene blue upregulates Nrf2/ARE genes and prevents tau-related neurotoxicity. Hum Mol Genet 2014,  23: 3716-3732. PMID: 24556215.



Dumont M, Stack C, Elipenahli C, Jainuddin S, Launay N, Gerges M, Starkova N, Starkov AA, Calingasan NY, Tampellini D, Pujol A, Beal MF.  PGC-1α overexpression exacerbates β-amyloid and tau deposition in a transgenic mouse model of Alzheimer’s disease.  FASEB J 2014, 28:1745-1755 PMID: 24398293.



Weiduschat N, Mao X, Beal MF, Nirenberg MJ, Shungu DC, Henchcliffe C.  Usefulness of Proton and Phosphorus MR Spectroscopic Imaging for Early Diagnosis of Parkinson’s Disease.  J. Neuroimaging 2013. PMID: 24325203




Boussicault L, Herard A-S, Caligasan N, Petit F, Malgorn C, Merienne N, Jan C, Gaillard

M-C, Cambon K, Lerchundi R, Barros LF, Escartin C, Delzescaux T, Mariani J, Gray, M,

Hantraye P, Beal, MF, Brouillet E, Vega C, Bonvento G.  Impaired brain energy

metabolism in Huntington Disease:  critical role of astrocyte-neuron interactions.  J. Cere

Blood Flow Metab.  2014, 34: 1500-1510 PMID 24938402