Manuscripts:

• Kayser EB, Chen Y, Mulholland M, Truong V, James K, Hanaford A, Johnson SC. Evaluating the efficacy of vatiquinone in preclinical models of mitochondrial disease. Preprint. June 2024. PMID: 38883711.

• Hanaford AR, Khanna A, James K, Truong V, Liao R, Chen Y, Mulholland M, Kayser EB, Watanabe K, Hsieh ES, Sedensky M, Morgan PG, Kalia V, Sarkar S, Johnson SC. Interferon-gamma contributes to disease progression in the Ndufs4(-/-) model of Leigh syndrome. Neuropathology and Applied Neurobiology. June 2024. PMID: 38680020.

• Spencer A K, Mulholland M, Snell J, Howe M, James K, Hanaford A, Morgan P, Sedensky M, Johnson SC. Volatile anaesthetic toxicity in the genetic mitochondrial disease Leigh syndrome. British Journal of Anaesthesia. Nov. 2023. PMID: 37770252.

• Bornstein R, Mulholland M, Sedensky M, Morgan M, Johnson SC. Glutamine metabolism in diseases associated with mitochondrial dysfunction. Molecular Cellular Neuroscience. Sept. 2023. PMID: 37586651.

• Hanaford A, Khanna A, James K, Chen Y, Mulholland M, Kayser B, Truong V, Sedensky M, Morgan P, Kalia V, Baerchst N, Sarkar S, Johnson SC: Peripheral macrophages causally contribute to disease onset and progression in the Ndufs4(KO) model of Leigh syndrome. Brain Pathology. Nov. 2023. PMID: 37552802.

• Spencer K, Woods C, Worstman H, Johnson SC, Ramirez J, Morgan P, Sedensky M. TREK-1 and TREK-2 knockout mice are not resistant to halothane or isoflurane. Anaesthesiology. April 2023. PMID: 37027798.

• Hanaford A, Johnson SC: The immune system as a driver of mitochondrial disease pathogenesis: a review of evidence. Orphanet Journal of Rare Diseases. Aug 15, 2022. PMID: 36056365.

• Stokes JC, Bornstein RL, James K, Park KY, Spencer K, Vo K, Snell JC, Johnson BM, Morgan PG, Sedensky MM, Baertsch N, Johnson SC: Leukocytes mediate disease pathogenesis in the Ndufs4(KO) mouse model of Leigh syndrome. JCI Insight. Jan 20, 2022. PMID: 35050903.

• Bornstein R, James K, Stokes J, Park KY, Snell J, Pan A, Bard A, Kalume F, Johnson SC: Differential effects of mTOR inhibition and dietary ketosis in a mouse model of subacute necrotizing encephalomyelopathy. Neurobiology of Disease. Dec 20, 2021. PMID: 34933094.

• Jung S, Kayser EB, Johnson SC, Li L, Worstman H, Sun G, Sedensky MM, Morgan PG: Tetraethylammonium chloride reduces anaesthetic-induced neurotoxicity in Caenorhabditis elegans and mice. British Journal of Anaesthesia. https://doi.org/10.1016/j.bja.2021.09.036, PMID: 34857359.

• Cheff DM, Muotri AR, Stockwell BR, Schmidt EE, Ran Q, Kartha RV, Johnson SC, Mittel P, Arner ESJ, Wigby KM, Hall MD, Rames SK: Development of Therapies for Rare Genetic Disorders of GPX4: Roadmap and Opportunities. Orphanet Journal of Rare Diseases. Epub 2021 Oct 23. PMID: 34688299.

• Stokes J, Freed A, Pan A, Sun G, Bornstein R, Snell J, Park KY, Morgan P, Sedensky MM, Johnson SC: Mechanisms underlying neonate specific metabolic effects of volatile anesthetics. eLIFE. Jul 12, 2021. PMID: 34254587.

• Bornstein R, Gonzalez B, Johnson SC: Mitochondrial pathways in human health and aging. Mitochondrion. 2020 Sep 54:72-84. DOI: 10.1016/j.mito.2020.07.007. Epub 2020 Jul 30. PMID: 32738358.

• Johnson SC, Kayser EB, Bornstein R, Stokes J, Bitto A, Park KY, Pan A, Sun G, Raftery D, Kaeberlein M, Sedensky MM, Morgan PG. Regional metabolic signatures in the Ndufs4(KO) mouse brain implicate defective glutamate/α-ketoglutarate metabolism in mitochondrial disease: Molecular Genetics and Metabolism. 2020 Jun;130(2):118-132. doi: 10.1016/j.ymgme.2020.03.007. April 3, 2020. PMID: 32331968

• 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. PLoS One. 2019. 14(3):e0214250. PMID: 30921410.

• 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. PLoS One. 2019. 14(3)e0213543. PMID: 30897103.

• 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. J Neurosurg Anesthesiol. 2019. 31(1):163-165. PMID: 30767942.

• Johnson, SC, Pan A, Li L, Sedensky M, Morgan P: Neurotoxicity of Anesthetics: Mechanisms and Meaning from Mouse Intervention Studies. Neurotoxicology and Teratology. 2019. 71:22-31. PMID: 30472095.

• 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 P, Sedensky M, Kaeberlein M, Legendre C, Suh Y, Canaud G: mTOR inhibitors may benefit kidney transplant recipients with mitochondrial diseases. Kidney International. 2019. 95(2):455-466. PMID: 30471880.

• Venot Q, Blanc T, Rabia S, Berteloot L, Ladraa S, Duong J, 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. Nature. 2018. 558:p540–546. PMID: 29899452.

• 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. Human Genetics. 2017. 136: p55-65. PMID: 27704213.

• Johnson SC, Kaeberlein M: Rapamycin in aging and disease: maximizing efficacy while minimizing side effects. Oncotarget. 2016; 7:p44876-44878. PMID: 27384492.

• 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 YI, 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 R, Völker U, Willems SM, Ohlsson C, Wallaschofski H, Kaplan R: Genomewide meta-analysis identifies loci associated with IGF-I and IGFBP-3 levels with impact on age-related traits. Aging Cell. 2016. 15(5):p811-24. PMID: 27329260.

• 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. Cell Metab. 2015. 22(5):p895-906. PMID: 26456335.

• 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. Front. Genet. 2015. 6:p247. PMID: 26257774.

• Johnson SC, Dong X, Vijg J, Suh Y: Genetic evidence for common pathways in human age-related diseases. Aging Cell. 2014 Oct;14(5):p809-17. PMID: 26077337.

• Johnson SC: Translational Medicine. A target for pharmacological intervention in an untreatable human disease. Science. 2014 Dec 5;346(6214). PMID: 25477449.

• 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. Plant Physiology. 165(1):p15-29. 2014. PMID: 24647477.

• 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. Science. 342(6165):p1524-8. 2013. PMID: 24231806.

• Johnson SC, Martin GM, Rabinovitch PS, Kaeberlein M: Preserving youth: does rapamycin deliver? Sci Transl Med. 5(211):p211fs40. 2013. PMID: 24225941.

• Johnson SC, Rabinovitch PS, Kaeberlein M: mTOR is a key modulator of ageing and age-related disease. Nature. 493(7432):p338-45. 2013. PMID: 23325216.

• 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. Int J Cancer. 134(4):p778-88. 2013. PMID: 23929039.

• Schleit J*, Johnson SC* (co-first), 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, 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, Wende HV, 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. Aging Cell. 12(6):p1050-61. 2012. PMID: 23837470.

• Johnson S, Nguyen V, Coder D: Assessment of cell viability. Curr Protoc Cytom. 9: 9.2. 2013. PMID: 23546778.

• 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. Front Genet. 4:129. 2013. PMC3701863.

• Johnson S, Rabinovitch P: Ex vivo imaging of excised tissue using vital dyes and confocal microscopy. Curr Protoc Cytom. 9:9.39. 2012. PMC3401092.

• Dai DF, Chen T, Johnson SC, Szeto H, Rabinovitch PS: Cardiac aging: from molecular mechanisms to significance in human health and disease. Antioxid Redox Signal. 15;16(12):1492-526. 2012. PMC3329953.

• Delaney JR, Sutphin GL, Dulken B, Sim S, Kim JR, Robinson B, Schleit J, Murakami CJ, 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. Aging Cell. 10(6)1089-91. 2011. PMC3215821.

• Dai DF, Johnson SC, Villarin JJ, Chin MT, Nieves-Clintron M, Chen T, Marcinek DJ, Dorn GW, Kang YJ, Prolla TA, Santana LF, Rabinovitch PS: Mitochondrial oxidative stress mediates angiotensin II-induced cardiac hypertrophy and Galphaq overexpression-induced heart failure. Circulation Research. 108(7):837-46. 2011. PMC3785241.