Simon C Johnson, PhD

Simon C Johnson, PhD

Mitochondrial Medicine and Metabolism Clinic

Academic Title: Assistant Professor, Neurology

Research Title: Principal Investigator, Seattle Children's Research Institute

Research Center: Center for Integrative Brain Research

  • Biography

    Dr. Simon Johnson is an assistant professor and principal investigator at the Center for Integrative Brain Research at Seattle Children’s Research Institute and an assistant professor of Neurology at the University of Washington. He earned his Bachelor’s degree in Biochemistry and Biophysics at Oregon State University, and his PhD in the Molecular Basis of Disease at the University of Washington. Following his graduate work, Simon trained as a post-doctoral fellow in Genetics at Albert Einstein College of Medicine in New York, before accepting a position at Seattle Children’s Research Institute. 

    The Simon Johnson laboratory studies the molecular and developmental mechanisms involved in the pathogenesis of pediatric genetic diseases, with a primary focus on mitochondrial disease. The aim of this work is to understand how genetic defects lead to the specific symptoms seen clinically, with the hope that a better understanding of disease pathways will lead to more effective and targeted therapies for pediatric genetic disorders.

    Simon joined Seattle Children’s Research Institute and the University of Washington with the belief that Seattle Children's Research Institute and UW are leading centers for mitochondrial disease research. Outside of work, Simon enjoys traveling, hiking, and backpacking trips with his wife. 

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  • Publications

    Other Publications

    • Johnson SC, Martinez F, Bitto A, Gonzalez B, Tazaerslan C, Cohen C, Delaval L, Timsit J, Knebelmann B, Terzi F, Mahal T, Zhu Y, Morgan PG, Sedensky MM, Kaeberlein M, Legendre C, Suh Y, Canaud G
      mTOR inhibitors may benefit kidney transplant recipients with mitochondrial diseases.
      30471880 Kidney international, 2019 Feb. : 95(2)455-466 PMCID:PMC6389356
    • Johnson SC, Pan A, Li L, Sedensky M, Morgan P
      Neurotoxicity of anesthetics: Mechanisms and meaning from mouse intervention studies.
      30472095 Neurotoxicology and teratology, 2019 Jan. : 7122-31 PMCID:PMC6330122
    • Johnson SC, Pan A, Sun GX, Freed A, Stokes JC, Bornstein R, Witkowski M, Li L, Ford JM, Howard CRA, Sedensky MM, Morgan PG
      Relevance of experimental paradigms of anesthesia induced neurotoxicity in the mouse.
      30897103 PloS one, 2019 : 14(3)e0213543 PMCID:PMC6428290
    • Kahn-Kirby AH, Amagata A, Maeder CI, Mei JJ, Sideris S, Kosaka Y, Hinman A, Malone SA, Bruegger JJ, Wang L, Kim V, Shrader WD, Hoff KG, Latham JC, Ashley EA, Wheeler MT, Bertini E, Carrozzo R, Martinelli D, Dionisi-Vici C, Chapman KA, Enns GM, Gahl W, Wolfe L, Saneto RP, Johnson SC, Trimmer JK, Klein MB, Holst CR
      Targeting ferroptosis: A novel therapeutic strategy for the treatment of mitochondrial disease-related epilepsy.
      30921410 PloS one, 2019 : 14(3)e0214250 PMCID:PMC6438538
    • Griffiths KK, Morgan PG, Johnson SC, Nambyiah P, Soriano SG, Johnson K, Xu J, Garber C, Maxwell L, Saraiya N
      A Summary of Preclinical Poster Presentations at the Sixth Biennial Pediatric Anesthesia Neurodevelopment Assessment (PANDA) Symposium.
      30767942 Journal of neurosurgical anesthesiology, 2019 Jan. : 31(1)163-165
    • Venot Q, Blanc T, Rabia SH, Berteloot L, Ladraa S, Duong JP, Blanc E, Johnson SC, Hoguin C, Boccara O, Sarnacki S, Boddaert N, Pannier S, Martinez F, Magassa S, Yamaguchi J, Knebelmann B, Merville P, Grenier N, Joly D, Cormier-Daire V, Michot C, Bole-Feysot C, Picard A, Soupre V, Lyonnet S, Sadoine J, Slimani L, Chaussain C, Laroche-Raynaud C, Guibaud L, Broissand C, Amiel J, Legendre C, Terzi F, Canaud G
      Targeted therapy in patients with PIK3CA-related overgrowth syndrome.
      29899452 Nature, 2018 June : 558(7711)540-546
    • Johnson SC
      Nutrient Sensing, Signaling and Ageing: The Role of IGF-1 and mTOR in Ageing and Age-Related Disease.
      30779006 Sub-cellular biochemistry, 2018 : 9049-97
    • Johnson SC, Gonzalez B, Zhang Q, Milholland B, Zhang Z, Suh Y
      Network analysis of mitonuclear GWAS reveals functional networks and tissue expression profiles of disease-associated genes.
      27704213 Human genetics, 2017 Jan. : 136(1)55-65 PMCID:PMC5214989
    • Teumer A, Qi Q, Nethander M, Aschard H, Bandinelli S, Beekman M, Berndt SI, Bidlingmaier M, Broer L, CHARGE Longevity Working Group., Cappola A, Ceda GP, Chanock S, Chen MH, Chen TC, Chen YD, Chung J, Del Greco Miglianico F, Eriksson J, Ferrucci L, Friedrich N, Gnewuch C, Goodarzi MO, Grarup N, Guo T, Hammer E, Hayes RB, Hicks AA, Hofman A, Houwing-Duistermaat JJ, Hu F, Hunter DJ, Husemoen LL, Isaacs A, Jacobs KB, Janssen JA, Jansson JO, Jehmlich N, Johnson S, Juul A, Karlsson M, Kilpelainen TO, Kovacs P, Kraft P, Li C, Linneberg A, Liu Y, Loos RJ, Body Composition Genetics Consortium., Lorentzon M, Lu Y, Maggio M, Magi R, Meigs J, Mellström D, Nauck M, Newman AB, Pollak MN, Pramstaller PP, Prokopenko I, Psaty BM, Reincke M, Rimm EB, Rotter JI, Saint Pierre A, Schurmann C, Seshadri S, Sjögren K, Slagboom PE, Strickler HD, Stumvoll M, Suh Y, Sun Q, Zhang C, Svensson J, Tanaka T, Tare A, Tönjes A, Uh HW, van Duijn CM, van Heemst D, Vandenput L, Vasan RS, Völker U, Willems SM, Ohlsson C, Wallaschofski H, Kaplan RC
      Genomewide meta-analysis identifies loci associated with IGF-I and IGFBP-3 levels with impact on age-related traits.
      27329260 Aging cell, 2016 Oct. : 15(5)811-24 PMCID:PMC5013013
    • Johnson SC, Kaeberlein M
      Rapamycin in aging and disease: maximizing efficacy while minimizing side effects.
      27384492 Oncotarget, 2016 Jul 19 : 7(29)44876-44878 PMCID:PMC5216691
    • McCormick MA, Delaney JR, Tsuchiya M, Tsuchiyama S, Shemorry A, Sim S, Chou AC, Ahmed U, Carr D, Murakami CJ, Schleit J, Sutphin GL, Wasko BM, Bennett CF, Wang AM, Olsen B, Beyer RP, Bammler TK, Prunkard D, Johnson SC, Pennypacker JK, An E, Anies A, Castanza AS, Choi E, Dang N, Enerio S, Fletcher M, Fox L, Goswami S, Higgins SA, Holmberg MA, Hu D, Hui J, Jelic M, Jeong KS, Johnston E, Kerr EO, Kim J, Kim D, Kirkland K, Klum S, Kotireddy S, Liao E, Lim M, Lin MS, Lo WC, Lockshon D, Miller HA, Moller RM, Muller B, Oakes J, Pak DN, Peng ZJ, Pham KM, Pollard TG, Pradeep P, Pruett D, Rai D, Robison B, Rodriguez AA, Ros B, Sage M, Singh MK, Smith ED, Snead K, Solanky A, Spector BL, Steffen KK, Tchao BN, Ting MK, Vander Wende H, Wang D, Welton KL, Westman EA, Brem RB, Liu XG, Suh Y, Zhou Z, Kaeberlein M, Kennedy BK
      A Comprehensive Analysis of Replicative Lifespan in 4,698 Single-Gene Deletion Strains Uncovers Conserved Mechanisms of Aging.
      26456335 Cell metabolism, 2015 Nov 3 : 22(5)895-906 PMCID:PMC4862740
    • Johnson SC, Dong X, Vijg J, Suh Y
      Genetic evidence for common pathways in human age-related diseases.
      26077337 Aging cell, 2015 Oct. : 14(5)809-17 PMCID:PMC4568968
    • Johnson SC, Yanos ME, Bitto A, Castanza A, Gagnidze A, Gonzalez B, Gupta K, Hui J, Jarvie C, Johnson BM, Letexier N, McCanta L, Sangesland M, Tamis O, Uhde L, Van Den Ende A, Rabinovitch PS, Suh Y, Kaeberlein M
      Dose-dependent effects of mTOR inhibition on weight and mitochondrial disease in mice.
      26257774 Frontiers in genetics, 2015 : 6247 PMCID:PMC4510413
    • Johnson SC, Sangesland M, Kaeberlein M, Rabinovitch PS
      Modulating mTOR in aging and health.
      25341517 Interdisciplinary topics in gerontology, 2015 : 40107-27
    • Johnson SC
      Translational Medicine. A target for pharmacological intervention in an untreatable human disease.
      25477449 Science (New York, N.Y.), 2014 Dec 5 : 346(6214)1192
    • Carbonell A, Takeda A, Fahlgren N, Johnson SC, Cuperus JT, Carrington JC
      New generation of artificial MicroRNA and synthetic trans-acting small interfering RNA vectors for efficient gene silencing in Arabidopsis.
      24647477 Plant physiology, 2014 May : 165(1)15-29 PMCID:PMC4012576
    • Wright JH, Johnson MM, Shimizu-Albergine M, Bauer RL, Hayes BJ, Surapisitchat J, Hudkins KL, Riehle KJ, Johnson SC, Yeh MM, Bammler TK, Beyer RP, Gilbertson DG, Alpers CE, Fausto N, Campbell JS
      Paracrine activation of hepatic stellate cells in platelet-derived growth factor C transgenic mice: evidence for stromal induction of hepatocellular carcinoma.
      23929039 International journal of cancer, 2014 Feb 15 : 134(4)778-88 PMCID:PMC3876966
    • Johnson SC, Yanos ME, Kayser EB, Quintana A, Sangesland M, Castanza A, Uhde L, Hui J, Wall VZ, Gagnidze A, Oh K, Wasko BM, Ramos FJ, Palmiter RD, Rabinovitch PS, Morgan PG, Sedensky MM, Kaeberlein M
      mTOR inhibition alleviates mitochondrial disease in a mouse model of Leigh syndrome.
      24231806 Science (New York, N.Y.), 2013 Dec 20 : 342(6165)1524-8 PMCID:PMC4055856
    • Schleit J, Johnson SC, Bennett CF, Simko M, Trongtham N, Castanza A, Hsieh EJ, Moller RM, Wasko BM, Delaney JR, Sutphin GL, Carr D, Murakami CJ, Tocchi A, Xian B, Chen W, Yu T, Goswami S, Higgins S, Holmberg M, Jeong KS, Kim JR, Klum S, Liao E, Lin MS, Lo W, Miller H, Olsen B, Peng ZJ, Pollard T, Pradeep P, Pruett D, Rai D, Ros V, Singh M, Spector BL, Vander Wende H, An EH, Fletcher M, Jelic M, Rabinovitch PS, MacCoss MJ, Han JD, Kennedy BK, Kaeberlein M
      Molecular mechanisms underlying genotype-dependent responses to dietary restriction.
      23837470 Aging cell, 2013 Dec. : 12(6)1050-61 PMCID:PMC3838465
    • Johnson SC, Martin GM, Rabinovitch PS, Kaeberlein M
      Preserving youth: does rapamycin deliver?
      24225941 Science translational medicine, 2013 Nov 13 : 5(211)211fs40 PMCID:PMC4019780
    • Johnson SC, Rabinovitch PS, Kaeberlein M
      mTOR is a key modulator of ageing and age-related disease.
      23325216 Nature, 2013 Jan 17 : 493(7432)338-45 PMCID:PMC3687363
    • Saha B, Zitnik G, Johnson S, Nguyen Q, Risques RA, Martin GM, Oshima J
      DNA damage accumulation and TRF2 degradation in atypical Werner syndrome fibroblasts with LMNA mutations.
      23847654 Frontiers in genetics, 2013 : 4129 PMCID:PMC3701863
    • Johnson S, Nguyen V, Coder D
      Assessment of cell viability.
      23546778 Current protocols in cytometry, 2013 : Chapter 9Unit9.2
    • Johnson S, Rabinovitch P
      Ex vivo imaging of excised tissue using vital dyes and confocal microscopy.
      22752953 Current protocols in cytometry, 2012 July : Chapter 9Unit 9.39 PMCID:PMC3401092
    • Dai DF, Chen T, Johnson SC, Szeto H, Rabinovitch PS
      Cardiac aging: from molecular mechanisms to significance in human health and disease.
      22229339 Antioxidants & redox signaling, 2012 Jun 15 : 16(12)1492-526 PMCID:PMC3329953
    • Delaney JR, Sutphin GL, Dulken B, Sim S, Kim JR, Robison B, Schleit J, Murakami CJ, Carr D, An EH, Choi E, Chou A, Fletcher M, Jelic M, Liu B, Lockshon D, Moller RM, Pak DN, Peng Q, Peng ZJ, Pham KM, Sage M, Solanky A, Steffen KK, Tsuchiya M, Tsuchiyama S, Johnson S, Raabe C, Suh Y, Zhou Z, Liu X, Kennedy BK, Kaeberlein M
      Sir2 deletion prevents lifespan extension in 32 long-lived mutants.
      21902802 Aging cell, 2011 Dec. : 10(6)1089-91 PMCID:PMC3215821
    • Dai DF, Johnson SC, Villarin JJ, Chin MT, Nieves-Cintrón M, Chen T, Marcinek DJ, Dorn GW 2nd, Kang YJ, Prolla TA, Santana LF, Rabinovitch PS
      Mitochondrial oxidative stress mediates angiotensin II-induced cardiac hypertrophy and Galphaq overexpression-induced heart failure.
      21311045 Circulation research, 2011 Apr 1 : 108(7)837-46 PMCID:PMC3785241

  • Research Funding

    Grant Title Grantor Amount Award Date
    Northwest Mitochondrial Research Guild Postdoctoral Fellowship Northwest Mitochondrial Research Guild Nov. 2016 - Sept. 2017
    Kirschstein-NRSA F32 Postdoctoral Fellowship NIH/NIA Sept. 2015 - Sept. 2017
    American Federation for Aging Research Postdoctoral Fellowship Award American Federation for Aging Sept. 2014 - Sept. 2015
    Mechanisms of Cardiovascular Disease NIH/NHLBI Sept. 2013 - Sept. 2014
    Genetic Approaches to Aging NIH/NIA Sept. 2009 - Aug. 2013