Publications
Defour, A., Medikayala, S., Van der Meulen, J. H., Hogarth, M. W., Holdreith, N., Malatras, A., Duddy, W., Boehler, J., Nagaraju, K., & Jaiswal, J. K. (2017). Annexin A2 links poor myofiber repair with inflammation and adipogenic replacement of the injured muscle. Human Molecular Genetics, 26(11), 1979–1991.
Dillingham, B. C., Knoblach, S. M., Many, G. M., Harmon, B. T., Mullen, A. M., Heier, C. R., Bello, L., McCall, J. M., Hoffman, E. P., Connor, E. M., & Nagaraju, K. (2014). VBP15, a Novel Anti-Inflammatory, is Effective at Reducing the Severity of Murine Experimental Autoimmune Encephalomyelitis. Cellular and Molecular Neurobiology, 35(3), 377-387.
Echigoya, Y., Rowel, K., Lim, Q., Trieu, N., Bao, B., Nichols, B. M., Vila, M. C., Novak, J. S., Hara, Y., Lee, J., Touznik, A., Mamchaoui, K., Aoki, Y., Takeda, S., Nagaraju, K., Mouly, V., Maruyama, R., Duddy, W., & Yokota, T. (2017). Quantitative Antisense Screening and Optimization for Exon 51 Skipping in Duchenne Muscular Dystrophy. Molecular Therapy, 25(11), 2561-2572.
Escolar, D. M., Zimmerman, A., Bertorini, T., Clemens, P. R., Connolly, A. M., Mesa, L., Gorni, K., Kornberg, A., Kolski, H., Kuntz, N., Nevo, Y., Tesi-Rocha, C., Nagaraju, K., Rayavarapu, S., Hache, L. P., Mayhew, J.E., Florence, J., Hu, F., Arrieta, A., Henricson, E., Leshner, R. T., & Mah, J. K. (2012). Pentoxifylline as a rescue treatment for DMD: A randomized double-blind clinical trial. Neurology, 78(12), 904–913.
Fiorillo, A. A., Tully, C. B., Damsker, J. M., Nagaraju, K., Hoffman, E. P., & Heier, C. R. (2018). Muscle miRNAome shows suppression of chronic inflammatory miRNAs with both prednisone and vamorolone. Physiological Genomics, 50(9), 735–745.
Freishtat, R. J., Mojgani, B., Nazemzadeh, M., Nagaraju, K., & Hoffman, E. P. (2007). NKG2A inhibits TH2 cell effector function in vitro. BMC Pulmonary Medicine, 7(1).
Freishtat, R. J., Nagaraju, K., Jusko, W., & Hoffman, E. P. (2010). Glucocorticoid Efficacy in Asthma. Journal of Investigative Medicine, 58(1), 19–22.
Fu, X., Brown, K. J., Rayavarapu, S., Nagaraju, K., & Liu, J. S. (2016). The use of proteomic analysis to study trafficking defects in axons. Methods in Cell Biology The Neuronal Cytoskeleton, Motor Proteins, and Organelle Trafficking in the Axon, 151–162.
Gordish-Dressman, H., Willmann, R., Pazze, L. D., Kreibich, A., Van Putten, M., Heydemann, A., Bogdanik, L., Lutz, C., Davies, K., Demonbruen, A. R., Duan, D., Elsey, D., Fukada, S., Girgenrath, M., Gonzalez, J. P., Groudns, M. D., Nichols, A., Partidge, T., Passini, M., Sanarica, F., Schnell, F. J., Wells, D. J., Yokota, T., Young, C. S., Zhong, Z., Spurney, C., Spencer, M., De Luca, A., Nagaraju, K., & Aartsma-Rus, A. (2018). “Of Mice and Measures”: A Project to Improve How We Advance Duchenne Muscular Dystrophy Therapies to the Clinic. Journal of Neuromuscular Diseases, 5(4), 407–417.
Grounds, M. D., Radley, H. G., Lynch, G. S., Nagaraju, K., & De Luca, A. (2008). Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy. Neurobiology of Disease, 31(1), 1–19.
Guerron, A. D., Rawat, R., Sali, A., Spurney, C. F., Pistilli, E., Cha, H.-J., Pandey, G. S., Gernapudi, R., Francia, D., Farajian, V., Escolar, D. M., Bossi, L., Becker, M., Zerr, P., de la Porte, S., Gordish-Dressman, H., Partridge, T., Hoffman, E. P., & Nagaraju, K. (2010). Functional and Molecular Effects of Arginine Butyrate and Prednisone on Muscle and Heart in the mdx Mouse Model of Duchenne Muscular Dystrophy. PLoS ONE, 5(6).
Hathout, Y., Marathi, R. L., Rayavarapu, S., Zhang, A., Brown, K. J., Seol, H., Gordish-Dressman, H., Cirak, S., Bello, L., Nagaraju, K., Partidge, T., Hoffman, E. P., Takeda, S., Mah, J. K., Henricson, & McDonald, C. (2014). Discovery of serum protein biomarkers in the mdx mouse model and cross-species comparison to Duchenne muscular dystrophy patients. Human Molecular Genetics, 23(24), 6458–6469.
Hathout, Y., Conklin, L. S., Seol, H., Gordish-Dressman, H., Brown, K. J., Morgenroth, L. P., Nagaraju, K., Heier, C. R., Damsker, J. M., van den Anker, J. N., Henricson, E., Clemens, P. R., Mah, J. K., McDonald, C., & Hoffman, E. P. (2016). Serum pharmacodynamic biomarkers for chronic corticosteroid treatment of children. Scientific Reports, 6(1).
Heier, C. R., Damsker, J. M., Yu, Q., Dillingham, B. C., Huynh, T., Van Der Meulen, J. H., Sali, A., Miller, B. K., Phadke, A., Scheffer, L., Quinn, J., Tatem, K., Jordan, S., Dadgar, S., Rodriguez, O. C., Albanese, C., Calhoun, M., Gordish-Dressman, H., Jaiswal, J. K., Connor, E. M., McCall, J. M., Hoffman, E. P., Reeves, E. K. M., & Nagaraju, K. (2013). VBP15, a novel anti‐inflammatory and membrane‐stabilizer, improves muscular dystrophy without side effects. EMBO Molecular Medicine, 5(10), 1569–1585.
Henriques-Pons, A., & Nagaraju, K. (2009). Nonimmune mechanisms of muscle damage in myositis: role of the endoplasmic reticulum stress response and autophagy in the disease pathogenesis. Current Opinion in Rheumatology, 21(6), 581–587.
Henriques-Pons, A., Yu, Q., Rayavarapu, S., Cohen, T. V., Ampong, B., Cha, H. J., Jahnke, V., Van der Meulen, J., Wang, D., Jiang, W., Kandimalla, E. R., Agrawal, S., Spurney, C. F., & Nagaraju, K. (2013). Role of toll-like receptors in the pathogenesis of dystrophin-deficient skeletal and heart muscle. Human Molecular Genetics, 23(10), 2604–2617.
Hoffman, E. P., Reeves, E., Damsker, J., Nagaraju, K., Mccall, J. M., Connor, E. M., & Bushby, K. (2012). Novel Approaches to Corticosteroid Treatment in Duchenne Muscular Dystrophy. Physical Medicine and Rehabilitation Clinics of North America, 23(4), 821–828.
Hoffman, E. P. (2016). Facilitating orphan drug development: Proceedings of the TREAT-NMD International Conference, December 2015, Washington, DC, USA. Workshop report/Neuromuscular Disorders, 27, 693-701.
Hoffman, E. P., Riddle, V., Siegler, M. A., Dickerson, D., Backonja, M., Kramer, W. G., Nagaraju, K., Gordish-Dressman, H., Damsker, J. M., & McCall, J. M. (2018). Phase 1 trial of vamorolone, a first-in-class steroid, shows improvements in side effects via biomarkers bridged to clinical outcomes. Steroids, 134, 43–52.
Howard, O. M. Z., Dong, H. F., Yang, D., Raben, N., Nagaraju, K., Rosen, A., Casciola-Rosen, L., Härtlein, M., Kron, M., Yang, D., Yiadom, K., Dwivedi, S., Plotz, P. H., & Oppenheim, J. J. (2002). Histidyl–tRNA Synthetase and Asparaginyl–tRNA Synthetase, Autoantigens in Myositis, Activate Chemokine Receptors on T Lymphocytes and Immature Dendritic Cells. The Journal of Experimental Medicine, 196(6), 781–791.
Humphrey, G. W., Mekhedov, E., Blank, P. S., De Morree, A., Pekkurnaz, G., Nagaraju, K., & Zimmerberg, J. (2012). GREG cells, a dysferlin-deficient myogenic mouse cell line. Experimental Cell Research, 318(2), 127–135.
Huynh, T., Uaesoontrachoon, K., Quinn, J. L., Tatem, K. S., Heier, C. R., Van Der Meulen, J. H., Yu, Q., Harris, M., Nolan, C. J., Haegeman, G., Grounds, M. D., & Nagaraju, K. (2013). Selective modulation through the glucocorticoid receptor ameliorates muscle pathology in mdx mice. The Journal of Pathology, 231(2), 223–235.
Jahnke, V. E., Van Der Meulen, J. H., Johnston, H. K., Ghimbovschi, S., Partridge, T., Hoffman, E. P., & Nagaraju, K. (2012). Metabolic remodeling agents show beneficial effects in the dystrophin-deficient mdx mouse model. Skeletal Muscle, 2(1), 16.
Jain, H. V., Boehler, J. F., Verthelyi, D., Nagaraju, K., & Beaucage, S. L. (2017). An amphipathic trans-acting phosphorothioate RNA element delivers an uncharged phosphorodiamidate morpholino sequence in mdx mouse myotubes. RSC Advances, 7(67), 42519–42528.
Kesari, A., Fukuda, M., Knoblach, S., Bashir, R., Nader, G. A., Rao, D., Nagaraju, K., & Hoffman, E. P. (2008). Dysferlin Deficiency Shows Compensatory Induction of Rab27A/Slp2a That May Contribute to Inflammatory Onset. The American Journal of Pathology, 173(5), 1476–1487.
Kinder, T. B., Rayavarapu, S., White, K., & Nagaraju, K. (2013). Pathogenesis of myositis: Lessons learned from animal studies. Indian Journal of Rheumatology, 8(4), 170–178.
Kornegay, J. N., Spurney, C. F., Nghiem, P. P., Brinkmeyer-Langford, C. L., Hoffman, E. P., & Nagaraju, K. (2014). Pharmacologic Management of Duchenne Muscular Dystrophy: Target Identification and Preclinical Trials. ILAR Journal, 55(1), 119–149.
Kostek, M. C., Nagaraju, K., Pistilli, E., Sali, A., Lai, S.-H., Gordon, B., & Chen, Y.-W. (2012). IL-6 signaling blockade increases inflammation but does not affect muscle function in the mdx mouse. BMC Musculoskeletal Disorders, 13(106).
Lamason, R., Zhao, P., Rawat, R., Davis, A., Hall, J. C., Chae, J. J., Agarwal, R., Cohen, P., Rosen, A., Hoffman, E. P., & Nagaraju, K. (2006). Sexual dimorphism in immune response genes as a function of puberty. BMC Immunology, 7(2).
Leikina, E., Defour, A., Melikov, K., Van Der Meulen, J. H., Nagaraju, K., Bhuvanendran, S., Gebert, C., Pfeifer, K., Chernomordik, L. V., & Jaiswal, J. K. (2015). Annexin A1 Deficiency does not Affect Myofiber Repair but Delays Regeneration of Injured Muscles. Scientific Reports, 5(1).
Li, C. K.-C., Knopp, P., Moncrieffe, H., Singh, B., Shah, S., Nagaraju, K., Varsani, H., Gao, B., & Wedderburn, L. R. (2009). Overexpression of MHC Class I Heavy Chain Protein in Young Skeletal Muscle Leads to Severe Myositis. The American Journal of Pathology, 175(3), 1030–1040.
Miller, F. W., Lamb, J. A., Schmidt, J., & Nagaraju, K. (2018). Risk factors and disease mechanisms in myositis. Nature Reviews Rheumatology, 14(5), 255–268.
Mintz, M., Vanderver, A., Brown, K. J., Lin, J., Wang, Z., Kaneski, C., Schiffmann, R., Nagaraju, K., Hoffman, E. P., & Hathout, Y. (2008). Time Series Proteome Profiling To Study Endoplasmic Reticulum Stress Response. Journal of Proteome Research, 7(6), 2435–2444.
Nader, G. A., Dastmalchi, M., Alexanderson, H., Grundtman, C., Gernapudi, R., Esbjörnsson, M., Wang, Z., Rönnelid, J., Hoffman, E. P., Nagaraju, K., & Lundberg, I. E. (2010). A Longitudinal, Integrated, Clinical, Histological and mRNA Profiling Study of Resistance Exercise in Myositis. Molecular Medicine, 16(11-12), 455–464.
Nagaraju, K., & Lundberg, I. E. (2011). Polymyositis and Dermatomyositis: Pathophysiology. Rheumatic Disease Clinincs of North America 37(2), 159-171
Nagaraju, K., Raben, N., Merritt, G., Loeffler, L., Kirk, K., & Plotz, P. (1998). A variety of cytokines and immunologically relevant surface molecules are expressed by normal human skeletal muscle cells under proinflammatory stimuli. Clinical and Experimental Immunology, 113(3), 407–414.
Nagaraju, K., Naik, S. R., & Naik, S. (1997). Functional implications of hepatitis B surface antigen (HBsAg) in the T cells of chronic HBV carriers. Journal of Viral Hepatitis, 4(4), 221–230.
Nagaraju, K., & Naik, S. (1995). Letter to the Editor. Clinical Immunology and Immunopathology, 77(2), 206-207.
Nagaraju, K., Raben, N., Loeffler, L., Parker, T., Rochon, P. J., Lee, E., … Plotz, P. (2000). Conditional up-regulation of MHC class I in skeletal muscle leads to self-sustaining autoimmune myositis and myositis-specific autoantibodies. Proceedings of the National Academy of Sciences, 97(16), 9209–9214.
Nagaraju, K. (2001). Immunological capabilities of skeletal muscle cells. Acta Physiologica Scandinavica, 171(3), 215–223.
Nagaraju, K., Raben, N., Villalba, M. L., Danning, C., Loeffler, L. A., Lee, E., … Plotz, P. H. (1999). Costimulatory Markers in Muscle of Patients with Idiopathic Inflammatory Myopathies and in Cultured Muscle Cells. Clinical Immunology, 92(2), 161–169.
Nagaraju, K., Casciola-Rosen, L., Rosen, A., Thompson, C., Loeffler, L., Parker, T., … Plotz, P. (2000). The Inhibition of Apoptosis in Myositis and in Normal Muscle Cells. The Journal of Immunology, 164(10), 5459–5465.
Nagaraju, K. (2001). Update on immunopathogenesis in inflammatory myopathies. Current Opinion in Rheumatology, 13(6), 461–468.
Nagaraju, K., & Plotz, P. H. (2002). Animal models of myositis. Rheumatic Disease Clinics of North America, 28(4), 917–933.
Nagaraju, K., Casciola-Rosen, L., Lundberg, I., Rawat, R., Cutting, S., Thapliyal, R., Chang, J., Dwivedi, S., Mitsak, M., Chen, Y-W., Plotz, P., Rosen, A., Hoffman, E., & Raben, N. (2005). Activation of the endoplasmic reticulum stress response in autoimmune myositis: Potential role in muscle fiber damage and dysfunction. Arthritis & Rheumatism, 52(6), 1824–1835.
Nagaraju, K. (2005). Role of major histocompatibility complex class I molecules in autoimmune myositis. Current Opinion in Rheumatology, 17(6), 725–730.
Nagaraju, K., Rider, L. G., Fan, C., Chen, Y-W., Mitsak, M., Rawat, R., Patterson, K., Grundtman, C., Miller, F. W., Plotz, P. H., Hoffman, E., & Lundberg, I. E. (2006) Endothelial cell activation and neovascularization are prominent in dermtomyositis. Journal of Autoimmune Diseases 3(2).
Nagaraju, K., Rawat, R., Veszelovszky, E., Thapliyal, R., Kesari, A., Sparks, S., Raben, N., Plotz, P., & Hoffman, E. P. (2008). Dysferlin Deficiency Enhances Monocyte Phagocytosis. The American Journal of Pathology, 172(3), 774–785.
Nagaraju, K., & Willmann, R. (2009). Developing standard procedures for murine and canine efficacy studies of DMD therapeutics: report of two expert workshops on “Pre-clinical testing for Duchenne dystrophy”: Washington DC, October 27th–28th 2007 and Zürich, June 30th-July 1st 2008. Neuromuscular Disorders, 19(7), 502–506.
Nagaraju, K., Ghimbovschi, S., Rayavarapu, S., Phadke, A., Rider, L. G., Hoffman, E. P., & Miller, F. W. (2016). Muscle myeloid type I interferon gene expression may predict therapeutic responses to rituximab in myositis patients. Rheumatology, 55(9), 1673–1680.
Nie, L., Chu, H., Cheng, Y., Spurney, C., Nagaraju, K., & Chen, J. (2009). Marginal and Conditional Approaches to Multivariate Variables Subject to Limit of Detection. Journal of Biopharmaceutical Statistics, 19(6), 1151–1161.
Novak, J. S., Hogarth, M. W., Boehler, J. F., Nearing, M., Vila, M. C., Heredia, R., Fiorillo, A. A., Zhang, A., Hathout, Y., Hoffman, E. P., Jaiswal, J. K., Nagaraju, K., Cirak, S., & Partridge, T. A. (2018). Author Correction: Myoblasts and macrophages are required for therapeutic morpholino antisense oligonucleotide delivery to dystrophic muscle. Nature Communications, 9(1).
Pandey, S. N., Cabotage, J., Shi, R., Dixit, M., Sutherland, M., Liu, J., Muger, S., Harper, S. Q., Nagaraju, K., & Chen, Y.-W. (2012). Conditional over-expression of PITX1 causes skeletal muscle dystrophy in mice. Biology Open, 1(7), 629–639.
Quinn, J. L., Huynh, T., Uaesoontrachoon, K., Tatem, K., Phadke, A., Van Der Meulen, J. H., Yu, Q., & Nagaraju, K. (2013). Effects of Dantrolene Therapy on Disease Phenotype in Dystrophin Deficient mdx Mice. PLoS Currents Muscular Dystrophy.
Raben, N., Danon, M., Gilbert, A. L., Dwivedi, S., Collins, B., Thurberg, B. L., Mattaliano, R. J., Nagaraju, K., & Plotz, P. (2003). Enzyme replacement therapy in the mouse model of Pompe disease. Molecular Genetics and Metabolism, 80(1-2), 159–169.
Raben, N., Lu, N., Nagaraju, K., Rivera, Y., Lee, A., Yan, B., Byrne, B., Meikle, P. J., Umapathysivam, K., Hopwood, J. J., & Plotz, P. (2001). Conditional tissue-specific expression of the acid alpha-glucosidase (GAA) gene in the GAA knockout mice: implications for therapy. Human Molecular Genetics, 10(19), 2039–2047.
Raben, N., Nagaraju, K., Lee, E., Kessler, P., Byrne, B., Lee, L., LaMarca, M., King, C., Ward, J., Sauer, B., & Plotz, P. (1998). Targeted Disruption of the Acid α-Glucosidase Gene in Mice Causes an Illness with Critical Features of Both Infantile and Adult Human Glycogen Storage Disease Type II. Journal of Biological Chemistry, 273(30), 19086–19092.
Raben, N., Nagaraju, K., Lee, E., & Plotz, P. (2000). Modulation of disease severity in mice with targeted disruption of the acid α-glucosidase gene. Neuromuscular Disorders, 10(4-5), 283–291.
Raben, N., Jatkar, T., Lee, A., Lu, N., Dwivedi, S., Nagaraju, K., & Plotz, P. H. (2002). Glycogen Stored in Skeletal but Not in Cardiac Muscle in Acid α-Glucosidase Mutant (Pompe) Mice Is Highly Resistant to Transgene-Encoded Human Enzyme. Molecular Therapy, 6(5), 601–608.
Raben, N., Nagaraju, K., Lee, A., Lu, N., Rivera, Y., Jatkar, T., Hopwood, J. J., & Plotz, P. H. (2003). Induction of tolerance to a recombinant human enzyme, acid alpha-glucosidase, in enzyme deficient knockout mice. Transgenic Research, 12, 171-178.
Raben, N., Fukuda, T., Gilbert, A. L., De Jong, D., Thurberg, B. L., Mattaliano, R. J., Meikle, P., Hopwood, J. J., Nagashima, K., Nagaraju, K., & Plotz, P. H. (2005). Replacing acid α-glucosidase in Pompe disease: recombinant and transgenic enzymes are equipotent, but neither completely clears glycogen from type II muscle fibers. Molecular Therapy, 11(1), 48–56.
Raben, N., Takikita, S., Pittis, M. G., Bembi, B., Marie, S. K. N., Roberts, A., Page, L., Kishnani, P. S., Schoser, B. G. H., Chien, Y-H., Ralston, E., Nagaraju, K., & Plotz, P. H. (2007). Deconstructing Pompe Disease by Analyzing Single Muscle Fibers: “To See a World in a Grain of Sand….” Autophagy, 3(6), 546–552.
Raben, N., Schreiner, C., Baum, R., Takikita, S., Xu, S., Xie, T., Myerowitz, R., Komatsu, M., Van Der Meulen, J. H., Nagaraju, K., Ralston, E., & Plotz, P. H. (2010). Suppression of autophagy permits successful enzyme replacement therapy in a lysosomal storage disorder—murine Pompe disease. Autophagy, 6(8), 1078–1089.
Rawat, R., Cohen, T. V., Ampong, B., Francia, D., Henriques-Pons, A., Hoffman, E. P., & Nagaraju, K. (2010). Inflammasome Up-Regulation and Activation in Dysferlin-Deficient Skeletal Muscle. The American Journal of Pathology, 176(6), 2891–2900.
Rayavarapu, S., Van der meulen, J. H., Gordish-Dressman, H., Hoffman, E. P., Nagaraju, K., & Knoblach, S. M. (2010). Characterization of Dysferlin Deficient SJL/J Mice to Assess Preclinical Drug Efficacy: Fasudil Exacerbates Muscle Disease Phenotype. PLoS ONE, 5(9).
Rayavarapu, S., Coley, W., & Nagaraju, K. (2011). An update on pathogenic mechanisms of inflammatory myopathies. Current Opinion in Rheumatology, 23(6), 579–584.
Rayavarapu, S., Coley, W., & Nagaraju, K. (2012). Endoplasmic Reticulum Stress in Skeletal Muscle Homeostasis and Disease. Current Rheumatology Reports, 14, 238–243.
Rayavarapu, S., Coley, W., Van der Meulen, J. H., Cakir, E., Tappeta, K., Kinder, T. B., Dillingham, C., Brown, K. J., Hathout, Y., & Nagaraju, K. (2013). Activation of the Ubiquitin Proteasome Pathway in a Mouse Model of Inflammatory Myopathy: A Potential Therapeutic Target. Arthritis & Rheumatism, 65(12), 3248–3258.
Rayavarapu, S., Coley, W., Cakir, E., Jahnke, V., Takeda, S., Aoki, Y., Gordish-Dressman, H., Jaiswal, J. K., Hoffman, E. P., Brown, K. J., Hat… Nagaraju, K. (2013). Identification of Disease Specific Pathways Using in Vivo SILAC Proteomics in Dystrophin DeficientmdxMouse. Molecular & Cellular Proteomics, 12(5), 1
Rayavarapu, S., Coley, W., Kinder, T. B., & Nagaraju, K. (2013). Idiopathic inflammatory myopathies: pathogenic mechanisms of muscle weakness. Skeletal Muscle, 3(1), 13.061–1073.
Reeves, E. K. M., Hoffman, E. P., Nagaraju, K., Damsker, J. M., & McCall, J. M. (2013). VBP15: Preclinical characterization of a novel anti-inflammatory delta 9,11 steroid. Bioorganic & Medicinal Chemistry, 21(8), 2241–2249.
Rosenberg, A. S., Puig, M., Nagaraju, K., Hoffman, E. P., Villalta, S. A., Rao, V. A., Wakefield, L. M., & Woodcock, J. (2015). Immune-mediated pathology in Duchenne muscular dystrophy. Science Translational Medicine, 7(299), 299rv4.
Sali, A., Guerron, A. D., Gordish-Dressman, H., Spurney, C. F., Iantorno, M., Hoffman, E. P., & Nagaraju, K. (2012). Glucocorticoid-Treated Mice Are an Inappropriate Positive Control for Long-Term Preclinical Studies in the mdx Mouse. PLoS ONE, 7(4).
Sali, A., Many, G. M., Gordish-Dressman, H., van der Meulen, J. H., Phadke, A., Spurney, C. F., Cnaan, A., Hoffman, E. P., & Nagaraju, K. (2013). The Proton Pump Inhibitor Lansoprazole Improves the Skeletal Phenotype in Dystrophin Deficient mdx Mice. PLoS ONE, 8(7).
Salomonsson, S., Grundtman, C., Zhang, S.-J., Lanner, J. T., Li, C., Katz, A., Wedderburn, L. R., Nagaraju, K., Lundberg, I. E., & Westerblad, H. (2009). Upregulation of MHC class I in transgenic mice results in reduced force-generating capacity in slow-twitch muscle. Muscle & Nerve, 39(5), 674–682.
Sharron, M., Hoptay, C. E., Wiles, A. A., Garvin, L. M., Geha, M., Benton, A. S., Nagaraju, K., & Freishtat, R. J. (2012). Platelets Induce Apoptosis during Sepsis in a Contact-Dependent Manner That Is Inhibited by GPIIb/IIIa Blockade. PLoS ONE, 7(7).
Shelton, G. D., Hoffman, E. P., Ghimbovschi, S., Peters, I. R., Day, M. J., Mullins, M., Moore, P. F., & Nagaraju, K. (2006). Immunopathogenic pathways in canine inflammatory myopathies resemble human myositis. Veterinary Immunology and Immunopathology, 113(1-2), 200–214.
Singh, V. K., & Nagaraju, K. (1996). Experimental autoimmune uveitis: Molecular mimicry and oral tolerance. Immunologic Research, 15(4), 323–346.
Spurney, C. F., Knoblach, S., Pistilli, E. E., Nagaraju, K., Martin, G. R., & Hoffman, E. P. (2008). Dystrophin-deficient cardiomyopathy in mouse: Expression of Nox4 and Lox are associated with fibrosis and altered functional parameters in the heart. Neuromuscular Disorders, 18(5), 371–381.
Spurney, C. F., Gordish-Dressman, H., Guerron, A. D., Sali, A., Pandey, G. S., Rawat, R., van der Meulen, J., Cha, H-J., Pistilli, E. E., Partridge, T. A., Hoffman, E. P., & Nagaraju, K. (2009). Preclinical drug trials in the mdx mouse: Assessment of reliable and sensitive outcome measures. Muscle & Nerve, 39(5), 591–602.
Spurney, C. F., Cha, H.-J., Sali, A., Pandey, G. S., Pistilli, E., Guerron, A. D., Gordish-Dressman, H., Hoffman, E. P., & Nagaraju, K. (2010). Evaluation of Skeletal and Cardiac Muscle Function after Chronic Administration of Thymosin β-4 in the Dystrophin Deficient Mouse. PLoS ONE, 5(1).
Spurney, C. F., Guerron, A. D., Yu, Q., Sali, A., van der Meulen, J. H., Hoffman, E. P., & Nagaraju, K. (2011). Membrane Sealant Poloxamer P188 Protects Against Isoproterenol Induced Cardiomyopathy in Dystrophin Deficient Mice. BMC Cardiovascular Disorders, 11(1).
Spurney, C. F., Sali, A., Guerron, A. D., Iantorno, M., Yu, Q., Gordish-Dressman, H., Rayavarapu, S., van der Meulen, J., Hoffman, E. P., & Nagaraju, K. (2011). Losartan Decreases Cardiac Muscle Fibrosis and Improves Cardiac Function in Dystrophin-Deficient Mdx Mice. Journal of Cardiovascular Pharmacology and Therapeutics, 16(1), 87–95.
Spurney, C., Yu, Q., & Nagaraju, K. (2011). Speckle tracking analysis of the left ventricular anterior wall shows significantly decreased relative radial strain patterns in dystrophin deficient mice after 9 months of age. PLoS Currents, 3.
Sreetama, S. C., Chandra, G., van der Meulen, J. H., Ahmad, M. M., Suzuki, P., Bhuvanendran, S., Nagaraju, K., Hoffman, E. P., & Jaiswal, J. K. (2018). Membrane Stabilization by Modified Steroid Offers a Potential Therapy for Muscular Dystrophy Due to Dysferlin Deficit. Molecular Therapy, 26(9), 2231–2242.
Takanohashi, A., Prust, M., Wang, J., Gordish-Dressman, H., Bloom, M., Rice, G. I., Schmidt, J. L., Crow, Y. J., Lebon, P., Kuijpers, T. W., Nagaraju, K., & Vanderver, A. (2013). Elevation of proinflammatory cytokines in patients with Aicardi-Goutieres syndrome. Neurology, 80(11), 997–1002.
Tezak, Z., Nagaraju, K., Plotz, P., & Hoffman, E. P. (2000). Adeno-associated virus in normal and myositis human skeletal muscle. Neurology, 55(12), 1913–1917.
Uaesoontrachoon, K., Quinn, J. L., Tatem, K. S., van der Meulen, J. H., Yu, Q., Phadke, A., Miller, B. K., Gordish-Dressman, H., Ongini, E., Miglietta, D., & Nagaraju, K. (2014). Long-term treatment with naproxcinod significantly improves skeletal and cardiac disease phenotype in the mdx mouse model of dystrophy. Human Molecular Genetics, 23(12), 3239–3249.
Uaesoontrachoon, K., Cha, H.-J., Ampong, B., Sali, A., Vandermeulen, J., Wei, B., Creeden, B., Huynh, T., Quinn, J., Tatem, K., Rayavarapu, S., Hoffman, E. P., & Nagaraju, K. (2013). The effects of MyD88 deficiency on disease phenotype in dysferlin-deficient A/J mice: role of endogenous TLR ligands. The Journal of Pathology, 231(2), 199–209.
Van Putten, M., Aartsma-Rus, A., Grounds, M. D., Kornegay, J. N., Mayhew, A., Gillingwater, T. H., Takeda, S., Ruegg, M. A., De Luca, A., Nagaraju, K., & Willmann, R. (2018). Update on Standard Operating Procedures in Preclinical Research for DMD and SMA Report of TREAT-NMD Alliance Workshop, Schiphol Airport, 26 April 2015, The Netherlands. Journal of Neuromuscular Diseases, 5(1), 29–34.
Varadkar, P., Despres, D., Kraman, M., Lozier, J., Phadke, A., Nagaraju, K., & Mccright, B. (2014). The protein phosphatase 2A B56γ regulatory subunit is required for heart development. Developmental Dynamics, 243(6), 778–790.
Vila, M. C., Klimek, M. B., Novak, J. S., Rayavarapu, S., Uaesoontrachoon, K., Boehler, J. F., Fiorillo, A. A., Hogarth, M. W., Zhang, A., Shaughnessy, C., Gordish-Dressman, H., Burki, U., Straub, V., Lu, Q. L., Partridge, T. A., Brown, K. J., Hathout, Y., van den Anker, J., Hoffman, E. P., & Nagaraju, K. (2015). Elusive sources of variability of dystrophin rescue by exon skipping. Skeletal Muscle, 5(44).
Wang, L., Almeida, L. E. F., Kamimura, S., van der Meulen, J. H., Nagaraju, K., Quezado, M., Wakim, P., & Quezado, Z. M. N. (2018). The role of nitrite in muscle function, susceptibility to contraction injury, and fatigability in sickle cell mice. Nitric Oxide, 80, 70–81.
Willmann, R., De Luca, A., Benatar, M., Grounds, M., Dubach, J., Raymackers, J.-M., & Nagaraju, K. (2012). Enhancing translation: Guidelines for standard pre-clinical experiments in mdx mice. Neuromuscular Disorders, 22(1), 43–49.
Willmann, R., Gordish-Dressman, H., Meinen, S., Rüegg, M. A., Yu, Q., Nagaraju, K., Kumar, A., Girgenrath, M., Coffey, C. B. M., Cruz, V., Van Ry, P. M., Bogdanik, L., Lutz, C., Rutkowski, A., & Burkin, D. J. (2017). Improving Reproducibility of Phenotypic Assessments in the DyW Mouse Model of Laminin-α2 Related Congenital Muscular Dystrophy. Journal of Neuromuscular Diseases, 4(2), 115–126.
Wu, B., Moulton, H. M., Iversen, P. L., Jiang, J., Li, J., Li, J., Spurney, C. F., Sali, A., Guerron, A. D., Nagaraju, K., Doran, T., Lu, P., Xiao, X., & Lu, Q. L. (2008). Effective rescue of dystrophin improves cardiac function in dystrophin-deficient mice by a modified morpholino oligomer. Proceedings of the National Academy of Sciences, 105(39), 14814–14819.
Wu, B., Xiao, B., Cloer, C., Shaban, M., Sali, A., Lu, P., Li, J., Nagaraju, K., Xiao, X., & Lu, Q. L. (2011). One-year Treatment of Morpholino Antisense Oligomer Improves Skeletal and Cardiac Muscle Functions in Dystrophic mdx Mice. Molecular Therapy, 19(3), 576–583.
Yokota, T., Pistilli, E., Duddy, W., & Nagaraju, K. (2007). Potential of oligonucleotide-mediated exon-skipping therapy for Duchenne muscular dystrophy. Expert Opinion on Biological Therapy, 7(6), 831–842.
Yu, Q., Sali, A., van der Meulen, J., Creeden, B. K., Gordish-Dressman, H., Rutkowski, A., Rayavarapu, S., Uaesoontrachoon, K., Huynh, T., Nagaraju, K., & Spurney, C. F. (2013). Omigapil Treatment Decreases Fibrosis and Improves Respiratory Rate in dy2J Mouse Model of Congenital Muscular Dystrophy. PLoS ONE, 8(6).
Yu, Q., Morales, M., Li, N., Fritz, A. G., Ruobing, R., Blaeser, A., Francois, E., Lu, Q.-L., Nagaraju, K., & Spurney, C. F. (2018). Skeletal, cardiac, and respiratory muscle function and histopathology in the P448Lneo− mouse model of FKRP-deficient muscular dystrophy. Skeletal Muscle, 8(1).
Zhang, A., Uaesoontrachoon, K., Shaughnessy, C., Das, J. R., Rayavarapu, S., Brown, K. J., Ray, P.E., Nagaraju, K., van den Anker, J. N., Hoffman, E. P., & Hathout, Y. (2015). The use of urinary and kidney SILAM proteomics to monitor kidney response to high dose morpholino oligonucleotides in the mdx mouse. Toxicology Reports, 2, 838–849.
