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Last Name
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David K. Simon, PH.D., M.D.

TitleAssociate Professor of Neurology
InstitutionBeth Israel Deaconess Medical Center
DepartmentNeurology
AddressBeth Israel Deaconess Medical Center
Neurology -CLS-638
330 Brookline Ave
Boston MA 02215
Phone617/735-3251
Fax617/735-3252

 Mentoring 
 current student opportunities
Available: 10/01/13, Expires: 09/30/15

Mitochondria are the “energy factories” of our cells and also a source of potentially damaging free radicals. Inherited mutations in mitochondrial DNA (mtDNA), which is inherited strictly along the maternal line, can cause various rare clinical disorders, with the brain being one of the most commonly affected organs. MtDNA mutations also may plan a role in more common disorders such as Parkinson’s disease. Somatic (acquired) mtDNA mutations may contribute to the process of aging itself. We are now studying mechanisms to eliminate mtDNA mutations from cells. To do this, we take advantage of the fact that many pathogenic mtDNA mutations are “heteroplasmic”, meaning that there is a mix of wild-type (normal) and mutant mtDNA within individual cells. We are manipulating molecular mechanisms that may contribute to selection for or against mutant mtDNA using pharmacologic and genetic strategies. Recent work on macroautophagy has identified “mitophagy” as an important mechanism for selectively eliminating dysfunctional mitochondria. We are testing the novel hypothesis that stimulating mitophagy by inhibiting mTOR kinase activity will enhance the preferential elimination of functionally deleterious mtDNA mutations in “cybrid” (cytoplasmic hybrid) cell lines harboring a heteroplasmic mtDNA mutation that is associated with a mitochondrial disorder in humans. We predict that enhancing mitophagy will lead to preferential elimination of mitochondria (or mitochondrial fragments) that are dysfunctional, which will tend to be those with relatively greater proportions of mutant mtDNA. This will lead to a shift over time towards a lower mutational burden. Converse experiments to inhibit mitophagy may lead to increased mutation levels. We also will test the impact of enhancing mitophagy in Polg “mutator” mice that express a proofreading deficient mitochondrial DNA polymerase resulting in an accelerated age-related accumulation of somatic mtDNA mutations and a premature aging phenotype. If our hypothesis proves to be correct, then clinical testing of strategies to enhance mitophagy can be foreseen in the future to promote a shift towards lower mutational burdens in patients with genetically based mitochondrial disorders. Similar strategies may be linked to aging and age-related neurodegenerative disease such as Parkinson’s disease. Start date and time commitment are flexible for this project. This project will involve tissue culture, western blots, quantitative PCR, immunocytochemistry, mouse models, cryostat sectioning, immunohistochemistry, cloning and sequencing, densitometry, and assays for mitochondrial function and for markers of oxidative stress. Prior laboratory research experience is preferred but not required.

Available: 07/23/12, Expires: 08/31/14

Epidemiological studies indicate that physical exercise is inversely related to the risk of Parkinson's disease (PD), raising the possibility that exercise has a neuroprotective effect on the brain. Modest exercise recently has been shown to slow the premature aging phenotype in "Polg mutator mice" that express a proofreading deficient form of Polg, resulting in the accumulation of somatic mitochondrial DNA (mtDNA) mutations. Exercise in these mice improves mitochondrial function, reduces brain atrophy, and extends lifespan. Exercise induces PGC-1alpha expression in muscle, and we hypothesize that it also may have this effect in the brain. PGC-1alpha is a transcriptional coactivator that stimulates mitochondrial biogensis and enhances antioxidant defenses. PGC-1alpha activity is low in the substantia nigra in the brain at very early stages of PD. We are now analyzing the brains of those mice to determine if exercise leads to a reduction in somatic mtDNA mutation accumulation, enhanced PGC-1alpha activity, and attenuation of the dopaminergic deficits that we have identified in these mice. If our hypothesis proves to be correct, then upregulation of PGC-1alpha in the brain might provide a mechanism linking exercise to neuroprotection against PD. This project will involve cryostat sectioning, immunohistochemistry, densitometry, quantitative PCR, western blots, and mitochondrial activity assays. Prior laboratory research is helpful but not required.

 completed student projects

 Bibliographic 
 selected publications
Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
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  1. Dai Y, Zheng K, Clark J, Swerdlow RH, Pulst SM, Sutton JP, Shinobu LA, Simon DK. Rapamycin drives selection against a pathogenic heteroplasmic mitochondrial DNA mutation. Hum Mol Genet. 2014 Feb 1; 23(3):637-47.
    View in: PubMed
  2. Dai Y, Kiselak T, Clark J, Clore E, Zheng K, Cheng A, Kujoth GC, Prolla TA, Maratos-Flier E, Simon DK. Behavioral and metabolic characterization of heterozygous and homozygous POLG mutator mice. Mitochondrion. 2013 Jul; 13(4):282-91.
    View in: PubMed
  3. Demetrius LA, Simon DK. The inverse association of cancer and Alzheimer's: a bioenergetic mechanism. J R Soc Interface. 2013; 10(82):20130006.
    View in: PubMed
  4. Clark J, Silvaggi JM, Kiselak T, Zheng K, Clore EL, Dai Y, Bass CE, Simon DK. Pgc-1a Overexpression Downregulates Pitx3 and Increases Susceptibility to MPTP Toxicity Associated with Decreased Bdnf. PLoS One. 2012; 7(11):e48925.
    View in: PubMed
  5. Demetrius LA, Simon DK. An inverse-Warburg effect and the origin of Alzheimer's disease. Biogerontology. 2012 Dec; 13(6):583-94.
    View in: PubMed
  6. Kumar KR, Weissbach A, Heldmann M, Kasten M, Tunc S, Sue CM, Svetel M, Kostic VS, Segura-Aguilar J, Ramirez A, Simon DK, Vieregge P, Münte TF, Hagenah J, Klein C, Lohmann K. Frequency of the D620N mutation in VPS35 in Parkinson disease. Arch Neurol. 2012 Oct; 69(10):1360-4.
    View in: PubMed
  7. Göbel A, Macklin EA, Winkler S, Betensky RA, Klein C, Lohmann K, Simon DK. Genetic risk factors in Parkinson's disease: single gene effects and interactions of genotypes. J Neurol. 2012 Nov; 259(11):2503-5.
    View in: PubMed
  8. 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 Jun; 71(6):850-4.
    View in: PubMed
  9. Shih LC, Piel J, Warren A, Kraics L, Silver A, Vanderhorst V, Simon DK, Tarsy D. Singing in groups for Parkinson's disease (SING-PD): a pilot study of group singing therapy for PD-related voice/speech disorders. Parkinsonism Relat Disord. 2012 Jun; 18(5):548-52.
    View in: PubMed
  10. Pankratz N, Beecham GW, DeStefano AL, Dawson TM, Doheny KF, Factor SA, Hamza TH, Hung AY, Hyman BT, Ivinson AJ, Krainc D, Latourelle JC, Clark LN, Marder K, Martin ER, Mayeux R, Ross OA, Scherzer CR, Simon DK, Tanner C, Vance JM, Wszolek ZK, Zabetian CP, Myers RH, Payami H, Scott WK, Foroud T. Meta-analysis of Parkinson's disease: identification of a novel locus, RIT2. Ann Neurol. 2012 Mar; 71(3):370-84.
    View in: PubMed
  11. Donovan S, Lim C, Rose P, Tarsy D, Sudarsky LR, Simon DK. The utility of laser-generated visual-cueing in Parkinsonian patients with gait freezing. Parkinsonism Relat Disord. 2012 May; 18(4):401.
    View in: PubMed
  12. Clark J, Reddy S, Zheng K, Betensky RA, Simon DK. Association of PGC-1alpha polymorphisms with age of onset and risk of Parkinson's disease. BMC Med Genet. 2011; 12:69.
    View in: PubMed
  13. Clark J, Dai Y, Simon DK. Do somatic mitochondrial DNA mutations contribute to Parkinson's disease? Parkinsons Dis. 2011; 2011:659694.
    View in: PubMed
  14. Xiao J, Zhao Y, Bastian RW, Perlmutter JS, Racette BA, Tabbal SD, Karimi M, Paniello RC, Wszolek ZK, Uitti RJ, Van Gerpen JA, Simon DK, Tarsy D, Hedera P, Truong DD, Frei KP, Blitzer A, Rudzinska M, Pfeiffer RF, Le C, LeDoux MS. The c.-237_236GA>TT THAP1 sequence variant does not increase risk for primary dystonia. Mov Disord. 2011 Feb 15; 26(3):549-52.
    View in: PubMed
  15. Weisskopf MG, Weuve J, Nie H, Saint-Hilaire MH, Sudarsky L, Simon DK, Hersh B, Schwartz J, Wright RO, Hu H. Association of cumulative lead exposure with Parkinson's disease. Environ Health Perspect. 2010 Nov; 118(11):1609-13.
    View in: PubMed
  16. Weaver VM, Kim NS, Jaar BG, Schwartz BS, Parsons PJ, Steuerwald AJ, Todd AC, Simon D, Lee BK. Associations of low-level urine cadmium with kidney function in lead workers. Occup Environ Med. 2011 Apr; 68(4):250-6.
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  17. Connolly BS, Feigenbaum AS, Robinson BH, Dipchand AI, Simon DK, Tarnopolsky MA. MELAS syndrome, cardiomyopathy, rhabdomyolysis, and autism associated with the A3260G mitochondrial DNA mutation. Biochem Biophys Res Commun. 2010 Nov 12; 402(2):443-7.
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  18. Clark J, Clore EL, Zheng K, Adame A, Masliah E, Simon DK. Oral N-acetyl-cysteine attenuates loss of dopaminergic terminals in alpha-synuclein overexpressing mice. PLoS One. 2010; 5(8):e12333.
    View in: PubMed
  19. Guo X, Kudryavtseva E, Bodyak N, Nicholas A, Dombrovsky I, Yang D, Kraytsberg Y, Simon DK, Khrapko K. Mitochondrial DNA deletions in mice in men: substantia nigra is much less affected in the mouse. Biochim Biophys Acta. 2010 Jun-Jul; 1797(6-7):1159-62.
    View in: PubMed
  20. Xiao J, Zhao Y, Bastian RW, Perlmutter JS, Racette BA, Tabbal SD, Karimi M, Paniell R, Blitzer A, Batish SD, Wszolek ZK, Uitti RJ, Van Gerpen JA, Hedera P, Simon DK, Tarsy D, Truong DD, Frei KP, Pfeiffer RF, Gong S, LeDoux MS. Novel human pathological mutations. Gene symbol: THAP1. Disease: dystonia 6. Hum Genet. 2010 Apr; 127(4):469.
    View in: PubMed
  21. Xiao J, Zhao Y, Bastian RW, Perlmutter JS, Racette BA, Tabbal SD, Karimi M, Paniello R, Blitzer A, Batish SD, Wszolek ZK, Uitti RJ, Van Gerpen JA, Hedera P, Simon DK, Tarsy D, Truong DD, Frei KP, Pfeiffer RF, Gong S, LeDoux MS. Novel human pathological mutations. Gene symbol: THAP1. Disease: dystonia 6. Hum Genet. 2010 Apr; 127(4):470.
    View in: PubMed
  22. Simon DK, Pankratz N, Kissell DK, Pauciulo MW, Halter CA, Rudolph A, Pfeiffer RF, Nichols WC, Foroud T. Maternal inheritance and mitochondrial DNA variants in familial Parkinson's disease. BMC Med Genet. 2010; 11:53.
    View in: PubMed
  23. Xiao J, Bastian RW, Perlmutter JS, Racette BA, Tabbal SD, Karimi M, Paniello R, Blitzer A, Batish SD, Wszolek ZK, Uitti RJ, Van Gerpen JA, Hedera P, Simon DK, Tarsy D, Truong DD, Frei KP, Pfeiffer RF, Gong S, LeDoux MS. Novel human pathological mutations. Gene symbol: THAP1. Disease: dystonia 6. Hum Genet. 2010 Apr; 127(4):470.
    View in: PubMed
  24. Xiao J, Zhao Y, Bastian RW, Perlmutter JS, Racette BA, Tabbal SD, Karimi M, Paniello R, Blitzer A, Batish SD, Wszolek ZK, Uitti RJ, Van Gerpen JA, Hedera P, Simon DK, Tarsy D, Truong DD, Frei KP, Pfeiffer RF, Gong S, LeDoux MS. Novel human pathological mutations. Gene symbol: THAP1. Disease: dystonia 6. Hum Genet. 2010 Apr; 127(4):469-70.
    View in: PubMed
  25. Xiao J, Zhao Y, Bastian RW, Perlmutter JS, Racette BA, Tabbal SD, Karimi M, Paniello R, Blitzer A, Batish SD, Wszolek ZK, Uitti RJ, Van Gerpen JA, Hedera P, Simon DK, Tarsy D, Truong DD, Frei KP, Pfeiffer RF, Gong S, LeDoux MS. Novel human pathological mutations. Gene symbol: THAP1. Disease: dystonia 6. Hum Genet. 2010 Apr; 127(4):470.
    View in: PubMed
  26. Xiao J, Zhao Y, Bastian RW, Perlmutter JS, Racette BA, Tabbal SD, Karimi M, Paniello RC, Wszolek ZK, Uitti RJ, Van Gerpen JA, Simon DK, Tarsy D, Hedera P, Truong DD, Frei KP, Dev Batish S, Blitzer A, Pfeiffer RF, Gong S, LeDoux MS. Novel THAP1 sequence variants in primary dystonia. Neurology. 2010 Jan 19; 74(3):229-38.
    View in: PubMed
  27. Simon D, Coyle M, Dagenais S, O'Neil J, Wai EK. Potential triaging of referrals for lumbar spinal surgery consultation: a comparison of referral accuracy from pain specialists, findings from advanced imaging and a 3-item questionnaire. Can J Surg. 2009 Dec; 52(6):473-80.
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  28. Sikorska M, Sandhu JK, Simon DK, Pathiraja V, Sodja C, Li Y, Ribecco-Lutkiewicz M, Lanthier P, Borowy-Borowski H, Upton A, Raha S, Pulst SM, Tarnopolsky MA. Identification of ataxia-associated mtDNA mutations (m.4052T>C and m.9035T>C) and evaluation of their pathogenicity in transmitochondrial cybrids. Muscle Nerve. 2009 Sep; 40(3):381-94.
    View in: PubMed
  29. Kraytsberg Y, Simon DK, Turnbull DM, Khrapko K. Do mtDNA deletions drive premature aging in mtDNA mutator mice? Aging Cell. 2009 Aug; 8(4):502-6.
    View in: PubMed
  30. Xiao J, Bastian RW, Perlmutter JS, Racette BA, Tabbal SD, Karimi M, Paniello RC, Blitzer A, Batish SD, Wszolek ZK, Uitti RJ, Hedera P, Simon DK, Tarsy D, Truong DD, Frei KP, Pfeiffer RF, Gong S, Zhao Y, LeDoux MS. High-throughput mutational analysis of TOR1A in primary dystonia. BMC Med Genet. 2009; 10:24.
    View in: PubMed
  31. Clark J, Simon DK. Transcribe to survive: transcriptional control of antioxidant defense programs for neuroprotection in Parkinson's disease. Antioxid Redox Signal. 2009 Mar; 11(3):509-28.
    View in: PubMed
  32. Simon, David K. Mitochondrial Encephalopathies. Encyclopedia of Movement Disorders. 2009.
  33. Simon, D.K. Parkinson's Disease. Textbook of Geriatrics and Gerontology, International. 2009; (In Press).
  34. Simon, David K. Complex I Deficiency in Parkinson's Disease. Encyclopedia of Movement Disorders. 2009.
  35. Tarsy D, Simon DK. Beating a dead horse: dopamine and Parkinson disease. Neurology. 2008 Nov 11; 71(20):1651; author reply 1651-2.
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  36. Simon DK, Swearingen CJ, Hauser RA, Trugman JM, Aminoff MJ, Singer C, Truong D, Tilley BC. Caffeine and progression of Parkinson disease. Clin Neuropharmacol. 2008 Jul-Aug; 31(4):189-96.
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  37. Sunita Venkateswaran, Mary Sacchetti, Doug Arnold, Dessa Sadovnick, Julia Kennedy; Donald Gagne, Brenda Banwell, the Canadian Pediatric Demyelinating Study Group, Amit Bar-Or and David Simon. Mitochondrial mutations in pediatric clinically isolated demyelinating syndromes (CIS). American Academy of Neurology Annual Meeting (Chicago; 2008). 2008; In Press.
  38. MG Weisskopf, J Weuve, H Nie, M Saint-Hilaire, L Sudarsky, DK Simon, B Hersh, J Schwartz, RO Wright, R Feldman, and H Hu. . Cumulative exposure to lead and risk of Parkinson’s disease. . 2008 International Society for Environmental Epidemiology (ISEE) Annual Meeting. 2008.
  39. David K. Simon. PD Monitor & Commentary. 2008.
  40. Wu AD, Fregni F, Simon DK, Deblieck C, Pascual-Leone A. Noninvasive brain stimulation for Parkinson's disease and dystonia. Neurotherapeutics. 2008 Apr; 5(2):345-61.
    View in: PubMed
  41. David K. Simon. Parkinson's Disease. Geriatrics Update. 2008.
  42. Lin MT, Cheng A, Zheng K, Atnip K, Kujoth G, Clark J., Arzberger T, Yang L, Betensky RA, Beal MF, Prolla TA, Standaert DG, Cantuti-Castelvetri I, Simon DK. . High Levels of Somatic Mitochondrial DNA Point Mutations in Early Parkinson’s Disease. American Neurological Association Annual Meeting (Washington D.C.). 2007.
  43. Djarmati A, Guzvic M, Grünewald A, Lang AE, Pramstaller PP, Simon DK, Kaindl AM, Vieregge P, Nygren AO, Beetz C, Hedrich K, Klein C. Rapid and reliable detection of exon rearrangements in various movement disorders genes by multiplex ligation-dependent probe amplification. Mov Disord. 2007 Sep 15; 22(12):1708-14.
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  44. Simon DK, Zheng K, Velázquez L, Santos N, Almaguer L, Figueroa KP, Pulst SM. Mitochondrial complex I gene variant associated with early age at onset in spinocerebellar ataxia type 2. Arch Neurol. 2007 Jul; 64(7):1042-4.
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  45. Lin MT, Cheng A, Zheng K, Atnip K, Kujoth G, Arzberger T, Yang L, Beal MF, Prolla TA, Standaert DG, Cantuti-Castelvetri I, Simon DK. . Somatic Mitochondrial DNA Point Mutations in Dopaminergic Neurons. American Academy of Neurology Annual Meeting (Boston). 2007.
  46. Joanne Clark DPhil; Michael A. Nili, PhD; Kangni Zheng MD; Shyan-Yuan Kao, PhD; Bruce A. Yankner, MD PhD; Glenn L. Wilson PhD; David K. Simon MD PhD. Rescue of alpha-synuclein induced glutathione depletion by n-acetylcysteine. American Academy of Neurology Annual Meeting (Boston). 2007.
  47. St-Pierre J, Drori S, Uldry M, Silvaggi JM, Rhee J, Jäger S, Handschin C, Zheng K, Lin J, Yang W, Simon DK, Bachoo R, Spiegelman BM. Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivators. Cell. 2006 Oct 20; 127(2):397-408.
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  48. Tarsy D, Simon DK. Dystonia. N Engl J Med. 2006 Aug 24; 355(8):818-29.
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  49. Fregni F, Simon DK, Wu A, Pascual-Leone A. Non-invasive brain stimulation for Parkinson's disease: a systematic review and meta-analysis of the literature. J Neurol Neurosurg Psychiatry. 2005 Dec; 76(12):1614-23.
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  50. Lee J, Kim CH, Simon DK, Aminova LR, Andreyev AY, Kushnareva YE, Murphy AN, Lonze BE, Kim KS, Ginty DD, Ferrante RJ, Ryu H, Ratan RR. Mitochondrial cyclic AMP response element-binding protein (CREB) mediates mitochondrial gene expression and neuronal survival. J Biol Chem. 2005 Dec 9; 280(49):40398-401.
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  51. Lin MT, Simon DK. No evidence for heritability of Parkinson disease in Swedish twins. Neurology. 2005 Mar 8; 64(5):932; author reply 932.
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  52. 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 Nov-Dec; 26(10):1343-55.
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  53. Simon DK, Nishino S, Scammell TE. Mistaken diagnosis of psychogenic gait disorder in a man with status cataplecticus ("limp man syndrome"). Mov Disord. 2004 Jul; 19(7):838-40.
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  54. Nichols WC, Uniacke SK, Pankratz N, Reed T, Simon DK, Halter C, Rudolph A, Shults CW, Conneally PM, Foroud T. Evaluation of the role of Nurr1 in a large sample of familial Parkinson's disease. Mov Disord. 2004 Jun; 19(6):649-55.
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  55. Tarnopolsky MA, Simon DK, Roy BD, Chorneyko K, Lowther SA, Johns DR, Sandhu JK, Li Y, Sikorska M. Attenuation of free radical production and paracrystalline inclusions by creatine supplementation in a patient with a novel cytochrome b mutation. Muscle Nerve. 2004 Apr; 29(4):537-47.
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  56. Tarnopolsky MA, Bourgeois JM, Fu MH, Kataeva G, Shah J, Simon DK, Mahoney D, Johns D, MacKay N, Robinson BH. Novel SCO2 mutation (G1521A) presenting as a spinal muscular atrophy type I phenotype. Am J Med Genet A. 2004 Mar 15; 125A(3):310-4.
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  57. Simon DK, Lin MT, Zheng L, Liu GJ, Ahn CH, Kim LM, Mauck WM, Twu F, Beal MF, Johns DR. Somatic mitochondrial DNA mutations in cortex and substantia nigra in aging and Parkinson's disease. Neurobiol Aging. 2004 Jan; 25(1):71-81.
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  58. Malouin R, Winch P, Leontsini E, Glass G, Simon D, Hayes EB, Schwartz BS. Longitudinal evaluation of an educational intervention for preventing tick bites in an area with endemic lyme disease in Baltimore County, Maryland. Am J Epidemiol. 2003 Jun 1; 157(11):1039-51.
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  59. Simon DK, Friedman J, Breakefield XO, Jankovic J, Brin MF, Provias J, Bressman SB, Charness ME, Tarsy D, Johns DR, Tarnopolsky MA. A heteroplasmic mitochondrial complex I gene mutation in adult-onset dystonia. Neurogenetics. 2003 Aug; 4(4):199-205.
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  60. Zheng K, Heydari B, Simon DK. A common NURR1 polymorphism associated with Parkinson disease and diffuse Lewy body disease. Arch Neurol. 2003 May; 60(5):722-5.
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  61. Simon, DK. Mitochondrial Encephalopathy, Lactic Acidosis and Stroke (MELAS). Encyclopedia of the Neurological Sciences; Eds: MJ Aminoff and RB Daroff. 2003.
  62. Simon, David K. Mitochondrial Mutations in Parkinson's Disease and Dystonias. Genetics of Movement Disorders; Ed: Stephan M. Pulst. 2003; 474 - 490.
  63. Simon DK, Lin MT, Pascual-Leone A. "Nature versus nurture" and incompletely penetrant mutations. J Neurol Neurosurg Psychiatry. 2002 Jun; 72(6):686-9.
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  64. Stewart WF, Schwartz BS, Simon D, Kelsey K, Todd AC. ApoE genotype, past adult lead exposure, and neurobehavioral function. Environ Health Perspect. 2002 May; 110(5):501-5.
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  65. Anschel DJ, Simon DK, Llinas R, Joseph JT. Spongiform encephalopathy mimicking corticobasal degeneration. Mov Disord. 2002 May; 17(3):606-7.
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  66. Lin MT, Simon DK, Ahn CH, Kim LM, Beal MF. High aggregate burden of somatic mtDNA point mutations in aging and Alzheimer's disease brain. Hum Mol Genet. 2002 Jan 15; 11(2):133-45.
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  67. Simon DK, Lin MT, Ahn CH, Liu GJ, Gibson GE, Beal MF, Johns DR. Low mutational burden of individual acquired mitochondrial DNA mutations in brain. Genomics. 2001 Apr 1; 73(1):113-6.
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  68. Simon DK, Tarnopolsky MA, Greenamyre JT, Johns DR. A frameshift mitochondrial complex I gene mutation in a patient with dystonia and cataracts: is the mutation pathogenic? J Med Genet. 2001 Jan; 38(1):58-61.
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  69. Simon DK, Beal MF. Pathogenesis: Oxidative Stress, Mitochondria Dysfunction and Excitotoxicity. Dis.: Dx and Clinical Management;Ch 27; Eds: SA Factor WJ Weiner. 2001.
  70. Tabamo RE, Fernandez HH, Friedman JH, Simon DK. Young-onset Parkinson's disease: a clinical pathologic description of two siblings. Mov Disord. 2000 Jul; 15(4):744-6.
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  71. Simon DK, Mayeux R, Marder K, Kowall NW, Beal MF, Johns DR. Mitochondrial DNA mutations in complex I and tRNA genes in Parkinson's disease. Neurology. 2000 Feb 8; 54(3):703-9.
    View in: PubMed
  72. Simon DK, Pulst SM, Sutton JP, Browne SE, Beal MF, Johns DR. Familial multisystem degeneration with parkinsonism associated with the 11778 mitochondrial DNA mutation. Neurology. 1999 Nov 10; 53(8):1787-93.
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  73. Simon DK. Parkinson disease in twins. JAMA. 1999 Oct 13; 282(14):1328; author reply 1328-9.
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  74. Simon DK, Standaert DG. Neuroprotective therapies. Med Clin North Am. 1999 Mar; 83(2):509-23, viii.
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  75. Simon DK, Johns DR. Mitochondrial disorders: clinical and genetic features. Annu Rev Med. 1999; 50:111-27.
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  76. Simon DK, Rodriguez ML, Frosch MP, Quackenbush EJ, Feske SK, Natowicz MR. A unique familial leukodystrophy with adult onset dementia and abnormal glycolipid storage: a new lysosomal disease? J Neurol Neurosurg Psychiatry. 1998 Aug; 65(2):251-4.
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  77. Wen PY, Alyea EP, Simon D, Herbst RS, Soiffer RJ, Antin JH. Guillain-Barré syndrome following allogeneic bone marrow transplantation. Neurology. 1997 Dec; 49(6):1711-4.
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  78. Simon DK, Roskies AL, O'Leary DD. Plasticity in the development of topographic order in the mammalian retinocollicular projection. Dev Biol. 1994 Apr; 162(2):384-93.
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  79. Simon DK, Prusky GT, O'Leary DD, Constantine-Paton M. N-methyl-D-aspartate receptor antagonists disrupt the formation of a mammalian neural map. Proc Natl Acad Sci U S A. 1992 Nov 15; 89(22):10593-7.
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  80. Simon DK, O'Leary DD. Responses of retinal axons in vivo and in vitro to position-encoding molecules in the embryonic superior colliculus. Neuron. 1992 Nov; 9(5):977-89.
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  81. Simon DK, O'Leary DD. Influence of position along the medial-lateral axis of the superior colliculus on the topographic targeting and survival of retinal axons. Brain Res Dev Brain Res. 1992 Oct 23; 69(2):167-72.
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  82. Simon DK, O'Leary DD. Development of topographic order in the mammalian retinocollicular projection. J Neurosci. 1992 Apr; 12(4):1212-32.
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  83. Simon DK, O'Leary DD. Relationship of retinotopic ordering of axons in the optic pathway to the formation of visual maps in central targets. J Comp Neurol. 1991 May 15; 307(3):393-404.
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  84. Simon DK, O'Leary DD. Limited topographic specificity in the targeting and branching of mammalian retinal axons. Dev Biol. 1990 Jan; 137(1):125-34.
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  85. Shapiro ML, Simon DK, Olton DS, Gage FH, Nilsson O, Björklund A. Intrahippocampal grafts of fetal basal forebrain tissue alter place fields in the hippocampus of rats with fimbria-fornix lesions. Neuroscience. 1989; 32(1):1-18.
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  86. Kleitman N, Simon DK, Schachner M, Bunge RP. Growth of embryonic retinal neurites elicited by contact with Schwann cell surfaces is blocked by antibodies to L1. Exp Neurol. 1988 Dec; 102(3):298-306.
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  87. Shapiro ML, Simon D, Olton DS, Gage FH, Bjorklund A, Stenevi U. Brain transplants: can they restore single unit activity in the hippocampus? Ann N Y Acad Sci. 1985; 444:536-7.
    View in: PubMed
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