Papers published in the last year: (chronologically)
•ACTA1 H40Y mutant iPSC-derived skeletal myocytes display mitochondrial defects in an in vitro model of nemaline myopathy. Gartz M, Haberman M, Sutton J, Slick RA, Luttrell SM, Mack DL, Lawlor MW. Exp Cell Res. 2023 Mar 15;424(2):113507. doi: 10.1016/j.yexcr.2023.113507. Epub 2023 Feb 14. PMID: 36796746
•Human Motor Neurons Elicit Pathological Hallmarks of ALS and Reveal Potential Biomarkers of the Disease in Response to Prolonged IFNγ Exposure. Chun C, Lee JH, Bothwell M, Nghiem P, Smith AST, Mack DL. J Neurosci. 2024 Apr 17;44(16):e1787232024. doi: 10.1523/ JNEUROSCI.1787-23.2024. PMID: 38413232
•Reversal of C9orf72 mutation-induced transcriptional dysregulation and pathology in cultured human neurons by allele-specific excision. Sachdev A, Gill K, Sckaff M, Birk AM, Aladesuyi Arogundade O, Brown KA, Chouhan RS, Issagholian-Lewin PO, Patel E, Watry HL, Bernardi MT, Keough KC, Tsai YC, Smith AST, Conklin BR, Clelland CD. Proc Natl Acad Sci U S A. 2024 Apr 23;121(17):e2307814121. doi: 10.1073/pnas.2307814121. Epub 2024 Apr 15. PMID: 38621131
•Incomplete-penetrant hypertrophic cardiomyopathy MYH7 G256E mutation causes hypercontractility and elevated mitochondrial respiration.. Lee S, Vander Roest AS, Blair CA, Kao K, Bremner SB, Childers MC, Pathak D, Heinrich P, Lee D, Chirikian O, Mohran SE, Roberts B, Smith JE, Jahng JW, Paik DT, Wu JC, Gunawardane RN, Ruppel KM, Mack DL, Pruitt BL, Regnier M, Wu SM, Spudich JA, Bernstein D. Proc Natl Acad Sci U S A. 2024 May 7;121(19):e2318413121. doi: 10.1073/pnas.2318413121. Epub 2024 Apr 29. PMID: 38683993
•Dystrophin deficiency impairs cell junction formation during embryonic myogenesis from pluripotent stem cells. Mozin E, Massouridès E, Mournetas V, Lièvre C, Bourdon A, Jackson DL, Packer JS, Seong J, Trapnell C, Le Guiner C, Adjali O, Pinset C, Mack DL, Dupont JB. iScience. 2024 Jun 11;27(7):110242. doi: 10.1016/j.isci.2024.110242. eCollection 2024 Jul 19.PMID: 39040067
•High mobility group box 1 (HMGB1) is a potential disease biomarker in cell and mouse models of Duchenne muscular dystrophy.. Slick RA, Sutton J, Haberman M, O’Brien BS, Tinklenberg JA, Mardikar A, Prom MJ, Beatka M, Gartz M, Vanden Avond MA, Siebers E, Mack DL, Gonzalez JP, Ebert AD, Nagaraju K, Lawlor MW. Biol Open. 2024 Sep 15;13(9):bio060542. doi: 10.1242/bio.060542. Epub 2024 Sep 5.PMID: 39158383
Chapters:
•Exploring the Potential for Biomaterials to Improve the Development of Spinal Motor Neurons from Induced Pluripotent Stem Cells. Seong, J., Chun, C., Smith, A.S.T, Joo J, Mack D.L. From the edited volume Motor Neurons – New Insights. Edited by Natalia Szejko. IntechOpen Press, London, UK. October 2023 DOI: 10.5772/intechopen. 113275.
Recent NDRG Publications (partial list)
1. Mair DB, Tsui JH, Higashi T, Koenig P, Dong Z, Chen JF, et al. Spaceflight-induced contractile and mitochondrial dysfunction in an automated heart-on-a-chip platform. Proc Natl Acad Sci USA. 2024 Oct;121(40):e2404644121.
2. Goldstein AJ, Padgett RM, Bremner SB, Higashi T, Obenaus AM, Bolduan S, et al. Engineered Heart Tissues for Standard 96-Well Tissue Culture Plates. Methods Mol Biol. 2024;2805:89–100.
3. Slick RA, Sutton J, Haberman M, O’Brien BS, Tinklenberg JA, Mardikar A, et al. High mobility group box 1 (HMGB1) is a potential disease biomarker in cell and mouse models of Duchenne muscular dystrophy. Biol Open. 2024 Sep 15;13(9).
4. Mozin E, Massouridès E, Mournetas V, Lièvre C, Bourdon A, Jackson DL, et al. Dystrophin deficiency impairs cell junction formation during embryonic myogenesis from pluripotent stem cells. iScience. 2024 Jul 19;27(7):110242.
5. Chirikian O, Faynus MA, Merk M, Singh Z, Muray C, Pham J, et al. YAP dysregulation triggers hypertrophy by CCN2 secretion and TGFβ uptake in human pluripotent stem cell-derived cardiomyocytes. BioRxiv. 2024 Jun 5;
6. Lee S, Vander Roest AS, Blair CA, Kao K, Bremner SB, Childers MC, et al. Incomplete-penetrant hypertrophic cardiomyopathy MYH7 G256E mutation causes hypercontractility and elevated mitochondrial respiration. Proc Natl Acad Sci USA. 2024 May 7;121(19):e2318413121.
7. Lee JD, Won W, Kimball K, Wang Y, Yeboah F, Evitts KM, et al. Structure-guided engineering of a fast genetically encoded sensor for real-time H2O2 monitoring. BioRxiv. 2024 Feb 4;
8. Mozin E, Massouridès E, Mournetas V, Lièvre C, Bourdon A, Jackson DL, et al. Dystrophin deficiency impairs cell junction formation during embryonic myogenesis. BioRxiv. 2024 Apr 8;
9. Gartz M, Haberman M, Sutton J, Slick RA, Luttrell SM, Mack DL, et al. ACTA1 H40Y mutant iPSC-derived skeletal myocytes display mitochondrial defects in an in vitro model of nemaline myopathy. Exp Cell Res. 2023 Mar 15;424(2):113507.
10. Pioner JM, Santini L, Palandri C, Langione M, Grandinetti B, Querceto S, et al. Calcium handling maturation and adaptation to increased substrate stiffness in human iPSC-derived cardiomyocytes: The impact of full-length dystrophin deficiency. Front Physiol. 2022 Nov 7;13:1030920.
11. Sachdev A, Gill K, Sckaff M, Birk AM, Aladesuyi Arogundade O, Brown KA, et al. Reversal of C9orf72 mutation-induced transcriptional dysregulation and pathology in cultured human neurons by allele-specific excision. Proc Natl Acad Sci USA. 2024 Apr 23;121(17):e2307814121.
12. Chun C, Lee JH, Bothwell M, Nghiem P, Smith AST, Mack DL. Human motor neurons elicit pathological hallmarks of ALS and reveal potential biomarkers of the disease in response to prolonged ifnγ exposure. J Neurosci. 2024 Apr 17;44(16).
13. Childers MC, Regnier M, Bothwell M, Smith AST. Conformational sampling of CMT-2D associated GlyRS mutations. Brain Multiphysics. 2022 Sep 6;3.
14. Bremner SB, Mandrycky CJ, Leonard A, Padgett RM, Levinson AR, Rehn ES, et al. Full-length dystrophin deficiency leads to contractile and calcium transient defects in human engineered heart tissues. J Tissue Eng. 2022 Dec;13:20417314221119628.
15. Shi Y, Liu G, Wu R, Mack DL, Sun XS, Maxwell J, et al. Differentiation Capacity of Human Urine-Derived Stem Cells to Retain Telomerase Activity. Front Cell Dev Biol. 2022 May 23;10:890574.
16. Bremner SB, Gaffney KS, Sniadecki NJ, Mack DL. A change of heart: human cardiac tissue engineering as a platform for drug development. Curr Cardiol Rep. 2022 May;24(5):473–486.
17. D’Elia AV, Tell G, Russo D, Arturi F, Puglisi F, Manfioletti G, et al. Expression and localization of the homeodomain-containing protein HEX in human thyroid tumors. J Clin Endocrinol Metab. 2002 Mar;87(3):1376–1383.
18. Boulanger CA, Mack DL, Booth BW, Smith GH. Interaction with the mammary microenvironment redirects spermatogenic cell fate in vivo. Proc Natl Acad Sci USA. 2007 Mar 6;104(10):3871–3876.
19. Fry ET, Mack DL, Sobel BE. The nature of synergy between tissue-type and single chain urokinase-type plasminogen activators. Thromb Haemost. 1989 Nov 24;62(3):909–916.
20. Smith AS, Luttrell SM, Dupont J-B, Gray K, Lih D, Fleming JW, et al. High-throughput, real-time monitoring of engineered skeletal muscle function using magnetic sensing. J Tissue Eng. 2022 Dec;13:20417314221122127.
21. Smith AST, McCain ML, Bothwell M, Mack DL. Editorial: Modeling neuromuscular diseases to determine molecular drivers of pathology and for drug discovery. Front Cell Dev Biol. 2022 Oct 5;10:1017356.
22. Smith AST, Kim JH, Chun C, Gharai A, Moon HW, Kim EY, et al. HDAC6 Inhibition Corrects Electrophysiological and Axonal Transport Deficits in a Human Stem Cell-Based Model of Charcot-Marie-Tooth Disease (Type 2D). Advanced Biology. 2022 Feb;6(2):e2101308.
23. Luttrell SM, Smith AST, Mack DL. Creating stem cell-derived neuromuscular junctions in vitro. Muscle Nerve. 2021 Oct;64(4):388–403.
24. Smith AST, Chun C, Hesson J, Mathieu J, Valdmanis PN, Mack DL, et al. Human Induced Pluripotent Stem Cell-Derived TDP-43 Mutant Neurons Exhibit Consistent Functional Phenotypes Across Multiple Gene Edited Lines Despite Transcriptomic and Splicing Discrepancies. Front Cell Dev Biol. 2021 Sep 29;9:728707.
25. Chang ACY, Pardon G, Chang ACH, Wu H, Ong S-G, Eguchi A, et al. Increased tissue stiffness triggers contractile dysfunction and telomere shortening in dystrophic cardiomyocytes. Stem Cell Rep. 2021 Sep 14;16(9):2169–2181.
26. Hamm SE, Fathalikhani DD, Bukovec KE, Addington AK, Zhang H, Perry JB, et al. Voluntary wheel running complements microdystrophin gene therapy to improve muscle function in mdx mice. Mol Ther Methods Clin Dev. 2021 Jun 11;21:144–160.
27. Mack DL, DaSilva AW, Rogers C, Hedlund E, Murphy EI, Vojdanovski V, et al. Mental Health and Behavior of College Students During the COVID-19 Pandemic: Longitudinal Mobile Smartphone and Ecological Momentary Assessment Study, Part II. J Med Internet Res. 2021 Jun 4;23(6):e28892.
28. Tsui JH, Leonard A, Camp ND, Long JT, Nawas ZY, Chavanachat R, et al. Tunable electroconductive decellularized extracellular matrix hydrogels for engineering human cardiac microphysiological systems. Biomaterials. 2021 May;272:120764.
29. Walker SJ, Wagoner AL, Leavitt D, Mack DL. A simplified approach for derivation of induced pluripotent stem cells from Epstein-Barr virus immortalized B-lymphoblastoid cell lines. Heliyon. 2021 Apr 3;7(4):e06617.
30. Chun C, Smith AST, Kim H, Kamenz DS, Lee JH, Lee JB, et al. Astrocyte-derived extracellular vesicles enhance the survival and electrophysiological function of human cortical neurons in vitro. Biomaterials. 2021 Apr;271:120700.
31. Klima JC, Doyle LA, Lee JD, Rappleye M, Gagnon LA, Lee MY, et al. Incorporation of sensing modalities into de novo designed fluorescence-activating proteins. Nat Commun. 2021 Feb 8;12(1):856.
32. Ross JA, Tasfaout H, Levy Y, Morgan J, Cowling BS, Laporte J, et al. rAAV-related therapy fully rescues myonuclear and myofilament function in X-linked myotubular myopathy. Acta Neuropathol Commun. 2020 Oct 19;8(1):167.
33. Choi JS, Smith AST, Williams NP, Matsubara T, Choi M, Kim J-W, et al. Nanopatterned Nafion microelectrode arrays for in vitro cardiac electrophysiology. Adv Funct Mater. 2020 Jun 18;30(25).
34. Williams NP, Rhodehamel M, Yan C, Smith AST, Jiao A, Murry CE, et al. Engineering anisotropic 3D tubular tissues with flexible thermoresponsive nanofabricated substrates. Biomaterials. 2020 May;240:119856.
35. Smith AST, Choi E, Gray K, Macadangdang J, Ahn EH, Clark EC, et al. NanoMEA: A Tool for High-Throughput, Electrophysiological Phenotyping of Patterned Excitable Cells. Nano Lett. 2020 Mar 11;20(3):1561–1570.
36. Najjar SA, Smith AST, Long CJ, McAleer CW, Cai Y, Srinivasan B, et al. A multiplexed in vitro assay system for evaluating human skeletal muscle functionality in response to drug treatment. Biotechnol Bioeng. 2020 Mar;117(3):736–747.
37. Willett NJ, Boninger ML, Miller LJ, Alvarez L, Aoyama T, Bedoni M, et al. Taking the next steps in regenerative rehabilitation: establishment of a new interdisciplinary field. Arch Phys Med Rehabil. 2020 Feb 5;
38. Dupont J-B, Guo J, Renaud-Gabardos E, Poulard K, Latournerie V, Lawlor MW, et al. AAV-Mediated Gene Transfer Restores a Normal Muscle Transcriptome in a Canine Model of X-Linked Myotubular Myopathy. Mol Ther. 2020 Feb 5;28(2):382–393.
39. Pioner JM, Guan X, Klaiman JM, Racca AW, Pabon L, Muskheli V, et al. Absence of full-length dystrophin impairs normal maturation and contraction of cardiomyocytes derived from human-induced pluripotent stem cells. Cardiovasc Res. 2020 Feb 1;116(2):368–382.
40. Bothwell M. Recent advances in understanding context-dependent mechanisms controlling neurotrophin signaling and function. [version 1; peer review: 3 approved]. F1000Res. 2019 Sep 19;8.
41. Bertero A, Fields PA, Smith AST, Leonard A, Beussman K, Sniadecki NJ, et al. Chromatin compartment dynamics in a haploinsufficient model of cardiac laminopathy. J Cell Biol. 2019 Sep 2;218(9):2919–2944.
42. Pioner JM, Santini L, Palandri C, Martella D, Lupi F, Langione M, et al. Optical Investigation of Action Potential and Calcium Handling Maturation of hiPSC-Cardiomyocytes on Biomimetic Substrates. Int J Mol Sci. 2019 Aug 3;20(15).
43. Berry BJ, Smith AST, Young JE, Mack DL. Advances and current challenges associated with the use of human induced pluripotent stem cells in modeling neurodegenerative disease. Cells Tissues Organs (Print). 2018 Oct 9;1–19.
44. Natarajan A, Smith AST, Berry B, Lambert S, Molnar P, Hickman JJ. Temporal characterization of neuronal migration behavior on chemically patterned neuronal circuits in a defined in vitro environment. ACS Biomater Sci Eng. 2018 Oct 8;4(10):3460–3470.
45. Berry BJ, Smith AST, Long CJ, Martin CC, Hickman JJ. Physiological Aβ Concentrations Produce a More Biomimetic Representation of the Alzheimer’s Disease Phenotype in iPSC Derived Human Neurons. ACS Chem Neurosci. 2018 Jul 18;9(7):1693–1701.
46. Jiao A, Moerk CT, Penland N, Perla M, Kim J, Smith AST, et al. Regulation of skeletal myotube formation and alignment by nanotopographically controlled cell-secreted extracellular matrix. J Biomed Mater Res A. 2018 Feb 19;106(6):1543–1551.
47. Murphy SV, Skardal A, Song L, Sutton K, Haug R, Mack DL, et al. Solubilized Amnion Membrane Hyaluronic Acid Hydrogel Accelerates Full-Thickness Wound Healing. Stem Cells Transl Med. 2017 Nov;6(11):2020–2032.
48. Mack DL. Reversion to embryonic transcriptional splicing patterns may underlie diabetic myopathy. Muscle Nerve. 2017 Aug 29;56(4):686–688.
49. Smith AST, Yoo H, Yi H, Ahn EH, Lee JH, Shao G, et al. Micro- and nano-patterned conductive graphene-PEG hybrid scaffolds for cardiac tissue engineering. Chem Commun. 2017 Jun 29;53(53):7412–7415.
50. Mack DL, Poulard K, Goddard MA, Latournerie V, Snyder JM, Grange RW, et al. Systemic AAV8-Mediated Gene Therapy Drives Whole-Body Correction of Myotubular Myopathy in Dogs. Mol Ther. 2017 Apr 5;25(4):839–854.
51. Mack DL, Cortés E, Giannini V, Török P, Roschuk T, Maier SA. Decoupling absorption and emission processes in super-resolution localization of emitters in a plasmonic hotspot. Nat Commun. 2017 Feb 17;8:14513.
52. Bothwell M. Mechanisms and medicines for remyelination. Annu Rev Med. 2017 Jan 14;68:431–443.
53. Smith AST, Macadangdang J, Leung W, Laflamme MA, Kim D-H. Human iPSC-derived cardiomyocytes and tissue engineering strategies for disease modeling and drug screening. Biotechnol Adv. 2017;35(1):77–94.
54. Smith AST, Passey SL, Martin NRW, Player DJ, Mudera V, Greensmith L, et al. Creating Interactions between Tissue-Engineered Skeletal Muscle and the Peripheral Nervous System. Cells Tissues Organs (Print). 2016 Nov 9;202(3-4):143–158.
55. Smith AST, Davis J, Lee G, Mack DL, Kim D-H. Muscular dystrophy in a dish: engineered human skeletal muscle mimetics for disease modeling and drug discovery. Drug Discov Today. 2016 Sep;21(9):1387–1398.
56. Carson D, Hnilova M, Yang X, Nemeth CL, Tsui JH, Smith AST, et al. Nanotopography-Induced Structural Anisotropy and Sarcomere Development in Human Cardiomyocytes Derived from Induced Pluripotent Stem Cells. ACS Appl Mater Interfaces. 2016 Aug 31;8(34):21923–21932.
57. Bothwell M. Recent advances in understanding neurotrophin signaling. [version 1; peer review: 2 approved]. F1000Res. 2016 Jul 28;5.
58. Pioner JM, Racca AW, Klaiman JM, Yang K-C, Guan X, Pabon L, et al. Isolation and Mechanical Measurements of Myofibrils from Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes. Stem Cell Rep. 2016 Jun 14;6(6):885–896.
59. Bothwell M. NGF, BDNF, NT3, and NT4. Handb Exp Pharmacol. 2014;220:3–15.
60. Guan X, Goddard MA, Mack DL, Childers MK. Gene therapy in monogenic congenital myopathies. Methods. 2016 Apr 15;99:91–98.
61. Mengsteab PY, Uto K, Smith AST, Frankel S, Fisher E, Nawas Z, et al. Spatiotemporal control of cardiac anisotropy using dynamic nanotopographic cues. Biomaterials. 2016 Apr;86:1–10.
62. Afzal MZ, Reiter M, Gastonguay C, McGivern JV, Guan X, Ge Z-D, et al. Nicorandil, a Nitric Oxide Donor and ATP-Sensitive Potassium Channel Opener, Protects Against Dystrophin-Deficient Cardiomyopathy. J Cardiovasc Pharmacol Ther. 2016 Mar 2;21(6):549–562.
63. Oleaga C, Bernabini C, Smith AST, Srinivasan B, Jackson M, McLamb W, et al. Multi-Organ toxicity demonstration in a functional human in vitro system composed of four organs. Sci Rep. 2016 Feb 3;6:20030.
64. Meabon JS, de Laat R, Ieguchi K, Serbzhinsky D, Hudson MP, Huber BR, et al. Intracellular LINGO-1 negatively regulates Trk neurotrophin receptor signaling. Mol Cell Neurosci. 2016 Jan;70:1–10.
65. Berry BJ, Akanda N, Smith AST, Long CJ, Schnepper MT, Guo X, et al. Morphological and functional characterization of human induced pluripotent stem cell-derived neurons (iCell Neurons) in defined culture systems. Biotechnol Prog. 2015 Dec;31(6):1613–1622.
66. Hyun YS, Lee J, Kim HJ, Hong YB, Koo H, Smith AST, et al. Charcot-Marie-Tooth Disease Type 4H Resulting from Compound Heterozygous Mutations in FGD4 from Nonconsanguineous Korean Families. Ann Hum Genet. 2015 Nov;79(6):460–469.
67. Goddard MA, Mack DL, Czerniecki SM, Kelly VE, Snyder JM, Grange RW, et al. Muscle pathology, limb strength, walking gait, respiratory function and neurological impairment establish disease progression in the p.N155K canine model of X-linked myotubular myopathy. Ann Transl Med. 2015 Oct;3(18):262.
68. Macadangdang J, Guan X, Smith AST, Lucero R, Czerniecki S, Childers MK, et al. Nanopatterned Human iPSC-based Model of a Dystrophin-Null Cardiomyopathic Phenotype. Cell Mol Bioeng. 2015 Sep;8(3):320–332.
69. Nam K-H, Smith AST, Lone S, Kwon S, Kim D-H. Biomimetic 3D Tissue Models for Advanced High-Throughput Drug Screening. J Lab Autom. 2015 Jun;20(3):201–215.
70. Chun SY, Mack DL, Moorefield E, Oh SH, Kwon TG, Pettenati MJ, et al. Pdx1 and controlled culture conditions induced differentiation of human amniotic fluid-derived stem cells to insulin-producing clusters. J Tissue Eng Regen Med. 2015 May;9(5):540–549.
71. Meabon JS, De Laat R, Ieguchi K, Wiley JC, Hudson MP, Bothwell M. LINGO-1 protein interacts with the p75 neurotrophin receptor in intracellular membrane compartments. J Biol Chem. 2015 Apr 10;290(15):9511–9520.
72. de Laat R, Meabon JS, Wiley JC, Hudson MP, Montine TJ, Bothwell M. LINGO-1 promotes lysosomal degradation of amyloid-β protein precursor. Pathobiol Aging Age Relat Dis. 2015 Mar 9;5:25796.
73. McAleer CW, Smith AST, Najjar S, Pirozzi K, Long CJ, Hickman JJ. Mechanistic investigation of adult myotube response to exercise and drug treatment in vitro using a multiplexed functional assay system. J Appl Physiol. 2014 Dec 1;117(11):1398–1405.
74. Mack DL, Guan X, Wagoner A, Walker SJ, Childers MK. Disease-in-a-dish: the contribution of patient-specific induced pluripotent stem cell technology to regenerative rehabilitation. Am J Phys Med Rehabil. 2014 Nov;93(11 Suppl 3):S155–68.
75. Braun R, Wang Z, Mack DL, Childers MK. Gene therapy for inherited muscle diseases: where genetics meets rehabilitation medicine. Am J Phys Med Rehabil. 2014 Nov;93(11 Suppl 3):S97–107.
76. Smith AST, Long CJ, McAleer C, Bobbitt N, Srinivasan B, Hickman JJ. Utilization of microscale silicon cantilevers to assess cellular contractile function in vitro. J Vis Exp. 2014 Oct 3;(92):e51866.
77. Smith AST, Long CJ, Pirozzi K, Najjar S, McAleer C, Vandenburgh HH, et al. A multiplexed chip-based assay system for investigating the functional development of human skeletal myotubes in vitro. J Biotechnol. 2014 Sep 20;185:15–18.
78. Wang Y, Sakano H, Beebe K, Brown MR, de Laat R, Bothwell M, et al. Intense and specialized dendritic localization of the fragile X mental retardation protein in binaural brainstem neurons: a comparative study in the alligator, chicken, gerbil, and human. J Comp Neurol. 2014 Jun 15;522(9):2107–2128.
79. Esch MB, Smith AST, Prot J-M, Oleaga C, Hickman JJ, Shuler ML. How multi-organ microdevices can help foster drug development. Adv Drug Deliv Rev. 2014 Apr;69-70:158–169.
80. Guan X, Mack DL, Moreno CM, Strande JL, Mathieu J, Shi Y, et al. Dystrophin-deficient cardiomyocytes derived from human urine: new biologic reagents for drug discovery. Stem Cell Res. 2014 Mar;12(2):467–480.
81. Childers MK, Joubert R, Poulard K, Moal C, Grange RW, Doering JA, et al. Gene therapy prolongs survival and restores function in murine and canine models of myotubular myopathy. Sci Transl Med. 2014 Jan 22;6(220):220ra10.
82. Smith AST, Long CJ, Berry BJ, McAleer C, Stancescu M, Molnar P, et al. Microphysiological systems and low-cost microfluidic platform with analytics. Stem Cell Res Ther. 2013 Dec 20;4 Suppl 1:S9.
83. Pirozzi KL, Long CJ, McAleer CW, Smith AST, Hickman JJ. Correlation of embryonic skeletal muscle myotube physical characteristics with contractile force generation on an atomic force microscope-based bio-microelectromechanical systems device. Appl Phys Lett. 2013 Aug 19;103(8):83108.
84. Padilla-Nash HM, McNeil NE, Yi M, Nguyen Q-T, Hu Y, Wangsa D, et al. Aneuploidy, oncogene amplification and epithelial to mesenchymal transition define spontaneous transformation of murine epithelial cells. Carcinogenesis. 2013 Aug;34(8):1929–1939.
85. Mirmalek-Sani S-H, Orlando G, McQuilling JP, Pareta R, Mack DL, Salvatori M, et al. Porcine pancreas extracellular matrix as a platform for endocrine pancreas bioengineering. Biomaterials. 2013 Jul;34(22):5488–5495.
86. Boulanger CA, Bruno RD, Mack DL, Gonzales M, Castro NP, Salomon DS, et al. Embryonic stem cells are redirected to non-tumorigenic epithelial cell fate by interaction with the mammary microenvironment. PLoS One. 2013 Apr 26;8(4):e62019.
87. Zhou Y, Mack DL, Williams JK, Mirmalek-Sani S-H, Moorefield E, Chun S-Y, et al. Genetic modification of primate amniotic fluid-derived stem cells produces pancreatic progenitor cells in vitro. Cells Tissues Organs (Print). 2013 Jan 8;197(4):269–282.
88. Smith AST, Long CJ, Pirozzi K, Hickman JJ. A functional system for high-content screening of neuromuscular junctions in vitro. Technology (Singap World Sci). 2013;1(1):37–48.
89. Schecterson LC, Sanchez JT, Rubel EW, Bothwell M. TrkB downregulation is required for dendrite retraction in developing neurons of chicken nucleus magnocellularis. J Neurosci. 2012 Oct 3;32(40):14000–14009.
90. Smith AST, Passey S, Greensmith L, Mudera V, Lewis MP. Characterization and optimization of a simple, repeatable system for the long term in vitro culture of aligned myotubes in 3D. J Cell Biochem. 2012 Mar;113(3):1044–1053.
91. Mudera V, Smith AST, Brady MA, Lewis MP. The effect of cell density on the maturation and contractile ability of muscle derived cells in a 3D tissue-engineered skeletal muscle model and determination of the cellular and mechanical stimuli required for the synthesis of a postural phenotype. J Cell Physiol. 2010 Nov;225(3):646–653.
92. Schecterson LC, Bothwell M. Neurotrophin receptors: Old friends with new partners. Dev Neurobiol. 2010 Apr;70(5):332–338.
93. Schecterson LC, Hudson MP, Ko M, Philippidou P, Akmentin W, Wiley J, et al. Trk activation in the secretory pathway promotes Golgi fragmentation. Mol Cell Neurosci. 2010 Apr;43(4):403–413.
94. Wiley JC, Meabon JS, Frankowski H, Smith EA, Schecterson LC, Bothwell M, et al. Phenylbutyric acid rescues endoplasmic reticulum stress-induced suppression of APP proteolysis and prevents apoptosis in neuronal cells. PLoS One. 2010 Feb 9;5(2):e9135.
95. Mandai K, Guo T, St Hillaire C, Meabon JS, Kanning KC, Bothwell M, et al. LIG family receptor tyrosine kinase-associated proteins modulate growth factor signals during neural development. Neuron. 2009 Sep 10;63(5):614–627.
96. Vilar M, Charalampopoulos I, Kenchappa RS, Simi A, Karaca E, Reversi A, et al. Activation of the p75 neurotrophin receptor through conformational rearrangement of disulphide-linked receptor dimers. Neuron. 2009 Apr 16;62(1):72–83.
97. Schecterson LC, Bothwell M. An all-purpose tool for axon guidance. Sci Signal. 2008 Nov 25;1(47):pe50.
98. Booth BW, Mack DL, Androutsellis-Theotokis A, McKay RDG, Boulanger CA, Smith GH. The mammary microenvironment alters the differentiation repertoire of neural stem cells. Proc Natl Acad Sci USA. 2008 Sep 30;105(39):14891–14896.
99. Strizzi L, Mancino M, Bianco C, Raafat A, Gonzales M, Booth BW, et al. Netrin-1 can affect morphogenesis and differentiation of the mouse mammary gland. J Cell Physiol. 2008 Sep;216(3):824–834.
100. Wiley JC, Smith EA, Hudson MP, Ladiges WC, Bothwell M. Fe65 stimulates proteolytic liberation of the beta-amyloid precursor protein intracellular domain. J Biol Chem. 2007 Nov 16;282(46):33313–33325.
101. Tesar PJ, Chenoweth JG, Brook FA, Davies TJ, Evans EP, Mack DL, et al. New cell lines from mouse epiblast share defining features with human embryonic stem cells. Nature. 2007 Jul 12;448(7150):196–199.
102. Mack DL, Boulanger CA, Callahan R, Smith GH. Expression of truncated Int6/eIF3e in mammary alveolar epithelium leads to persistent hyperplasia and tumorigenesis. Breast Cancer Res. 2007;9(4):R42.
103. Hatch NE, Hudson M, Seto ML, Cunningham ML, Bothwell M. Intracellular retention, degradation, and signaling of glycosylation-deficient FGFR2 and craniosynostosis syndrome-associated FGFR2C278F. J Biol Chem. 2006 Sep 15;281(37):27292–27305.
104. Bothwell M. Evolution of the neurotrophin signaling system in invertebrates. Brain Behav Evol. 2006 Aug 14;68(3):124–132.
105. Wiley JC, Hudson M, Kanning KC, Schecterson LC, Bothwell M. Familial Alzheimer’s disease mutations inhibit gamma-secretase-mediated liberation of beta-amyloid precursor protein carboxy-terminal fragment. J Neurochem. 2005 Sep;94(5):1189–1201.
106. Hatch NE, Nociti F, Swanson E, Bothwell M, Somerman M. FGF2 alters expression of the pyrophosphate/phosphate regulating proteins, PC-1, ANK and TNAP, in the calvarial osteoblastic cell line, MC3T3E1(C4). Connect Tissue Res. 2005;46(4-5):184–192.
107. Kanning KC, Hudson M, Amieux PS, Wiley JC, Bothwell M, Schecterson LC. Proteolytic processing of the p75 neurotrophin receptor and two homologs generates C-terminal fragments with signaling capability. J Neurosci. 2003 Jul 2;23(13):5425–5436.
1. Mair DB, Tsui JH, Higashi T, Koenig P, Dong Z, Chen JF, et al. Spaceflight-induced contractile and mitochondrial dysfunction in an automated heart-on-a-chip platform. Proc Natl Acad Sci USA. 2024 Oct;121(40):e2404644121.
2. Goldstein AJ, Padgett RM, Bremner SB, Higashi T, Obenaus AM, Bolduan S, et al. Engineered Heart Tissues for Standard 96-Well Tissue Culture Plates. Methods Mol Biol. 2024;2805:89–100.
3. Slick RA, Sutton J, Haberman M, O’Brien BS, Tinklenberg JA, Mardikar A, et al. High mobility group box 1 (HMGB1) is a potential disease biomarker in cell and mouse models of Duchenne muscular dystrophy. Biol Open. 2024 Sep 15;13(9).
4. Mozin E, Massouridès E, Mournetas V, Lièvre C, Bourdon A, Jackson DL, et al. Dystrophin deficiency impairs cell junction formation during embryonic myogenesis from pluripotent stem cells. iScience. 2024 Jul 19;27(7):110242.
5. Chirikian O, Faynus MA, Merk M, Singh Z, Muray C, Pham J, et al. YAP dysregulation triggers hypertrophy by CCN2 secretion and TGFβ uptake in human pluripotent stem cell-derived cardiomyocytes. BioRxiv. 2024 Jun 5;
6. Lee S, Vander Roest AS, Blair CA, Kao K, Bremner SB, Childers MC, et al. Incomplete-penetrant hypertrophic cardiomyopathy MYH7 G256E mutation causes hypercontractility and elevated mitochondrial respiration. Proc Natl Acad Sci USA. 2024 May 7;121(19):e2318413121.
7. Lee JD, Won W, Kimball K, Wang Y, Yeboah F, Evitts KM, et al. Structure-guided engineering of a fast genetically encoded sensor for real-time H2O2 monitoring. BioRxiv. 2024 Feb 4;
8. Mozin E, Massouridès E, Mournetas V, Lièvre C, Bourdon A, Jackson DL, et al. Dystrophin deficiency impairs cell junction formation during embryonic myogenesis. BioRxiv. 2024 Apr 8;
9. Gartz M, Haberman M, Sutton J, Slick RA, Luttrell SM, Mack DL, et al. ACTA1 H40Y mutant iPSC-derived skeletal myocytes display mitochondrial defects in an in vitro model of nemaline myopathy. Exp Cell Res. 2023 Mar 15;424(2):113507.
10. Pioner JM, Santini L, Palandri C, Langione M, Grandinetti B, Querceto S, et al. Calcium handling maturation and adaptation to increased substrate stiffness in human iPSC-derived cardiomyocytes: The impact of full-length dystrophin deficiency. Front Physiol. 2022 Nov 7;13:1030920.
11. Sachdev A, Gill K, Sckaff M, Birk AM, Aladesuyi Arogundade O, Brown KA, et al. Reversal of C9orf72 mutation-induced transcriptional dysregulation and pathology in cultured human neurons by allele-specific excision. Proc Natl Acad Sci USA. 2024 Apr 23;121(17):e2307814121.
12. Chun C, Lee JH, Bothwell M, Nghiem P, Smith AST, Mack DL. Human motor neurons elicit pathological hallmarks of ALS and reveal potential biomarkers of the disease in response to prolonged ifnγ exposure. J Neurosci. 2024 Apr 17;44(16).
13. Childers MC, Regnier M, Bothwell M, Smith AST. Conformational sampling of CMT-2D associated GlyRS mutations. Brain Multiphysics. 2022 Sep 6;3.
14. Bremner SB, Mandrycky CJ, Leonard A, Padgett RM, Levinson AR, Rehn ES, et al. Full-length dystrophin deficiency leads to contractile and calcium transient defects in human engineered heart tissues. J Tissue Eng. 2022 Dec;13:20417314221119628.
15. Shi Y, Liu G, Wu R, Mack DL, Sun XS, Maxwell J, et al. Differentiation Capacity of Human Urine-Derived Stem Cells to Retain Telomerase Activity. Front Cell Dev Biol. 2022 May 23;10:890574.
16. Bremner SB, Gaffney KS, Sniadecki NJ, Mack DL. A change of heart: human cardiac tissue engineering as a platform for drug development. Curr Cardiol Rep. 2022 May;24(5):473–486.
17. D’Elia AV, Tell G, Russo D, Arturi F, Puglisi F, Manfioletti G, et al. Expression and localization of the homeodomain-containing protein HEX in human thyroid tumors. J Clin Endocrinol Metab. 2002 Mar;87(3):1376–1383.
18. Boulanger CA, Mack DL, Booth BW, Smith GH. Interaction with the mammary microenvironment redirects spermatogenic cell fate in vivo. Proc Natl Acad Sci USA. 2007 Mar 6;104(10):3871–3876.
19. Fry ET, Mack DL, Sobel BE. The nature of synergy between tissue-type and single chain urokinase-type plasminogen activators. Thromb Haemost. 1989 Nov 24;62(3):909–916.
20. Smith AS, Luttrell SM, Dupont J-B, Gray K, Lih D, Fleming JW, et al. High-throughput, real-time monitoring of engineered skeletal muscle function using magnetic sensing. J Tissue Eng. 2022 Dec;13:20417314221122127.
21. Smith AST, McCain ML, Bothwell M, Mack DL. Editorial: Modeling neuromuscular diseases to determine molecular drivers of pathology and for drug discovery. Front Cell Dev Biol. 2022 Oct 5;10:1017356.
22. Smith AST, Kim JH, Chun C, Gharai A, Moon HW, Kim EY, et al. HDAC6 Inhibition Corrects Electrophysiological and Axonal Transport Deficits in a Human Stem Cell-Based Model of Charcot-Marie-Tooth Disease (Type 2D). Advanced Biology. 2022 Feb;6(2):e2101308.
23. Luttrell SM, Smith AST, Mack DL. Creating stem cell-derived neuromuscular junctions in vitro. Muscle Nerve. 2021 Oct;64(4):388–403.
24. Smith AST, Chun C, Hesson J, Mathieu J, Valdmanis PN, Mack DL, et al. Human Induced Pluripotent Stem Cell-Derived TDP-43 Mutant Neurons Exhibit Consistent Functional Phenotypes Across Multiple Gene Edited Lines Despite Transcriptomic and Splicing Discrepancies. Front Cell Dev Biol. 2021 Sep 29;9:728707.
25. Chang ACY, Pardon G, Chang ACH, Wu H, Ong S-G, Eguchi A, et al. Increased tissue stiffness triggers contractile dysfunction and telomere shortening in dystrophic cardiomyocytes. Stem Cell Rep. 2021 Sep 14;16(9):2169–2181.
26. Hamm SE, Fathalikhani DD, Bukovec KE, Addington AK, Zhang H, Perry JB, et al. Voluntary wheel running complements microdystrophin gene therapy to improve muscle function in mdx mice. Mol Ther Methods Clin Dev. 2021 Jun 11;21:144–160.
27. Mack DL, DaSilva AW, Rogers C, Hedlund E, Murphy EI, Vojdanovski V, et al. Mental Health and Behavior of College Students During the COVID-19 Pandemic: Longitudinal Mobile Smartphone and Ecological Momentary Assessment Study, Part II. J Med Internet Res. 2021 Jun 4;23(6):e28892.
28. Tsui JH, Leonard A, Camp ND, Long JT, Nawas ZY, Chavanachat R, et al. Tunable electroconductive decellularized extracellular matrix hydrogels for engineering human cardiac microphysiological systems. Biomaterials. 2021 May;272:120764.
29. Walker SJ, Wagoner AL, Leavitt D, Mack DL. A simplified approach for derivation of induced pluripotent stem cells from Epstein-Barr virus immortalized B-lymphoblastoid cell lines. Heliyon. 2021 Apr 3;7(4):e06617.
30. Chun C, Smith AST, Kim H, Kamenz DS, Lee JH, Lee JB, et al. Astrocyte-derived extracellular vesicles enhance the survival and electrophysiological function of human cortical neurons in vitro. Biomaterials. 2021 Apr;271:120700.
31. Klima JC, Doyle LA, Lee JD, Rappleye M, Gagnon LA, Lee MY, et al. Incorporation of sensing modalities into de novo designed fluorescence-activating proteins. Nat Commun. 2021 Feb 8;12(1):856.
32. Ross JA, Tasfaout H, Levy Y, Morgan J, Cowling BS, Laporte J, et al. rAAV-related therapy fully rescues myonuclear and myofilament function in X-linked myotubular myopathy. Acta Neuropathol Commun. 2020 Oct 19;8(1):167.
33. Choi JS, Smith AST, Williams NP, Matsubara T, Choi M, Kim J-W, et al. Nanopatterned Nafion microelectrode arrays for in vitro cardiac electrophysiology. Adv Funct Mater. 2020 Jun 18;30(25).
34. Williams NP, Rhodehamel M, Yan C, Smith AST, Jiao A, Murry CE, et al. Engineering anisotropic 3D tubular tissues with flexible thermoresponsive nanofabricated substrates. Biomaterials. 2020 May;240:119856.
35. Smith AST, Choi E, Gray K, Macadangdang J, Ahn EH, Clark EC, et al. NanoMEA: A Tool for High-Throughput, Electrophysiological Phenotyping of Patterned Excitable Cells. Nano Lett. 2020 Mar 11;20(3):1561–1570.
36. Najjar SA, Smith AST, Long CJ, McAleer CW, Cai Y, Srinivasan B, et al. A multiplexed in vitro assay system for evaluating human skeletal muscle functionality in response to drug treatment. Biotechnol Bioeng. 2020 Mar;117(3):736–747.
37. Willett NJ, Boninger ML, Miller LJ, Alvarez L, Aoyama T, Bedoni M, et al. Taking the next steps in regenerative rehabilitation: establishment of a new interdisciplinary field. Arch Phys Med Rehabil. 2020 Feb 5;
38. Dupont J-B, Guo J, Renaud-Gabardos E, Poulard K, Latournerie V, Lawlor MW, et al. AAV-Mediated Gene Transfer Restores a Normal Muscle Transcriptome in a Canine Model of X-Linked Myotubular Myopathy. Mol Ther. 2020 Feb 5;28(2):382–393.
39. Pioner JM, Guan X, Klaiman JM, Racca AW, Pabon L, Muskheli V, et al. Absence of full-length dystrophin impairs normal maturation and contraction of cardiomyocytes derived from human-induced pluripotent stem cells. Cardiovasc Res. 2020 Feb 1;116(2):368–382.
40. Bothwell M. Recent advances in understanding context-dependent mechanisms controlling neurotrophin signaling and function. [version 1; peer review: 3 approved]. F1000Res. 2019 Sep 19;8.
41. Bertero A, Fields PA, Smith AST, Leonard A, Beussman K, Sniadecki NJ, et al. Chromatin compartment dynamics in a haploinsufficient model of cardiac laminopathy. J Cell Biol. 2019 Sep 2;218(9):2919–2944.
42. Pioner JM, Santini L, Palandri C, Martella D, Lupi F, Langione M, et al. Optical Investigation of Action Potential and Calcium Handling Maturation of hiPSC-Cardiomyocytes on Biomimetic Substrates. Int J Mol Sci. 2019 Aug 3;20(15).
43. Berry BJ, Smith AST, Young JE, Mack DL. Advances and current challenges associated with the use of human induced pluripotent stem cells in modeling neurodegenerative disease. Cells Tissues Organs (Print). 2018 Oct 9;1–19.
44. Natarajan A, Smith AST, Berry B, Lambert S, Molnar P, Hickman JJ. Temporal characterization of neuronal migration behavior on chemically patterned neuronal circuits in a defined in vitro environment. ACS Biomater Sci Eng. 2018 Oct 8;4(10):3460–3470.
45. Berry BJ, Smith AST, Long CJ, Martin CC, Hickman JJ. Physiological Aβ Concentrations Produce a More Biomimetic Representation of the Alzheimer’s Disease Phenotype in iPSC Derived Human Neurons. ACS Chem Neurosci. 2018 Jul 18;9(7):1693–1701.
46. Jiao A, Moerk CT, Penland N, Perla M, Kim J, Smith AST, et al. Regulation of skeletal myotube formation and alignment by nanotopographically controlled cell-secreted extracellular matrix. J Biomed Mater Res A. 2018 Feb 19;106(6):1543–1551.
47. Murphy SV, Skardal A, Song L, Sutton K, Haug R, Mack DL, et al. Solubilized Amnion Membrane Hyaluronic Acid Hydrogel Accelerates Full-Thickness Wound Healing. Stem Cells Transl Med. 2017 Nov;6(11):2020–2032.
48. Mack DL. Reversion to embryonic transcriptional splicing patterns may underlie diabetic myopathy. Muscle Nerve. 2017 Aug 29;56(4):686–688.
49. Smith AST, Yoo H, Yi H, Ahn EH, Lee JH, Shao G, et al. Micro- and nano-patterned conductive graphene-PEG hybrid scaffolds for cardiac tissue engineering. Chem Commun. 2017 Jun 29;53(53):7412–7415.
50. Mack DL, Poulard K, Goddard MA, Latournerie V, Snyder JM, Grange RW, et al. Systemic AAV8-Mediated Gene Therapy Drives Whole-Body Correction of Myotubular Myopathy in Dogs. Mol Ther. 2017 Apr 5;25(4):839–854.
51. Mack DL, Cortés E, Giannini V, Török P, Roschuk T, Maier SA. Decoupling absorption and emission processes in super-resolution localization of emitters in a plasmonic hotspot. Nat Commun. 2017 Feb 17;8:14513.
52. Bothwell M. Mechanisms and medicines for remyelination. Annu Rev Med. 2017 Jan 14;68:431–443.
53. Smith AST, Macadangdang J, Leung W, Laflamme MA, Kim D-H. Human iPSC-derived cardiomyocytes and tissue engineering strategies for disease modeling and drug screening. Biotechnol Adv. 2017;35(1):77–94.
54. Smith AST, Passey SL, Martin NRW, Player DJ, Mudera V, Greensmith L, et al. Creating Interactions between Tissue-Engineered Skeletal Muscle and the Peripheral Nervous System. Cells Tissues Organs (Print). 2016 Nov 9;202(3-4):143–158.
55. Smith AST, Davis J, Lee G, Mack DL, Kim D-H. Muscular dystrophy in a dish: engineered human skeletal muscle mimetics for disease modeling and drug discovery. Drug Discov Today. 2016 Sep;21(9):1387–1398.
56. Carson D, Hnilova M, Yang X, Nemeth CL, Tsui JH, Smith AST, et al. Nanotopography-Induced Structural Anisotropy and Sarcomere Development in Human Cardiomyocytes Derived from Induced Pluripotent Stem Cells. ACS Appl Mater Interfaces. 2016 Aug 31;8(34):21923–21932.
57. Bothwell M. Recent advances in understanding neurotrophin signaling. [version 1; peer review: 2 approved]. F1000Res. 2016 Jul 28;5.
58. Pioner JM, Racca AW, Klaiman JM, Yang K-C, Guan X, Pabon L, et al. Isolation and Mechanical Measurements of Myofibrils from Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes. Stem Cell Rep. 2016 Jun 14;6(6):885–896.
59. Bothwell M. NGF, BDNF, NT3, and NT4. Handb Exp Pharmacol. 2014;220:3–15.
60. Guan X, Goddard MA, Mack DL, Childers MK. Gene therapy in monogenic congenital myopathies. Methods. 2016 Apr 15;99:91–98.
61. Mengsteab PY, Uto K, Smith AST, Frankel S, Fisher E, Nawas Z, et al. Spatiotemporal control of cardiac anisotropy using dynamic nanotopographic cues. Biomaterials. 2016 Apr;86:1–10.
62. Afzal MZ, Reiter M, Gastonguay C, McGivern JV, Guan X, Ge Z-D, et al. Nicorandil, a Nitric Oxide Donor and ATP-Sensitive Potassium Channel Opener, Protects Against Dystrophin-Deficient Cardiomyopathy. J Cardiovasc Pharmacol Ther. 2016 Mar 2;21(6):549–562.
63. Oleaga C, Bernabini C, Smith AST, Srinivasan B, Jackson M, McLamb W, et al. Multi-Organ toxicity demonstration in a functional human in vitro system composed of four organs. Sci Rep. 2016 Feb 3;6:20030.
64. Meabon JS, de Laat R, Ieguchi K, Serbzhinsky D, Hudson MP, Huber BR, et al. Intracellular LINGO-1 negatively regulates Trk neurotrophin receptor signaling. Mol Cell Neurosci. 2016 Jan;70:1–10.
65. Berry BJ, Akanda N, Smith AST, Long CJ, Schnepper MT, Guo X, et al. Morphological and functional characterization of human induced pluripotent stem cell-derived neurons (iCell Neurons) in defined culture systems. Biotechnol Prog. 2015 Dec;31(6):1613–1622.
66. Hyun YS, Lee J, Kim HJ, Hong YB, Koo H, Smith AST, et al. Charcot-Marie-Tooth Disease Type 4H Resulting from Compound Heterozygous Mutations in FGD4 from Nonconsanguineous Korean Families. Ann Hum Genet. 2015 Nov;79(6):460–469.
67. Goddard MA, Mack DL, Czerniecki SM, Kelly VE, Snyder JM, Grange RW, et al. Muscle pathology, limb strength, walking gait, respiratory function and neurological impairment establish disease progression in the p.N155K canine model of X-linked myotubular myopathy. Ann Transl Med. 2015 Oct;3(18):262.
68. Macadangdang J, Guan X, Smith AST, Lucero R, Czerniecki S, Childers MK, et al. Nanopatterned Human iPSC-based Model of a Dystrophin-Null Cardiomyopathic Phenotype. Cell Mol Bioeng. 2015 Sep;8(3):320–332.
69. Nam K-H, Smith AST, Lone S, Kwon S, Kim D-H. Biomimetic 3D Tissue Models for Advanced High-Throughput Drug Screening. J Lab Autom. 2015 Jun;20(3):201–215.
70. Chun SY, Mack DL, Moorefield E, Oh SH, Kwon TG, Pettenati MJ, et al. Pdx1 and controlled culture conditions induced differentiation of human amniotic fluid-derived stem cells to insulin-producing clusters. J Tissue Eng Regen Med. 2015 May;9(5):540–549.
71. Meabon JS, De Laat R, Ieguchi K, Wiley JC, Hudson MP, Bothwell M. LINGO-1 protein interacts with the p75 neurotrophin receptor in intracellular membrane compartments. J Biol Chem. 2015 Apr 10;290(15):9511–9520.
72. de Laat R, Meabon JS, Wiley JC, Hudson MP, Montine TJ, Bothwell M. LINGO-1 promotes lysosomal degradation of amyloid-β protein precursor. Pathobiol Aging Age Relat Dis. 2015 Mar 9;5:25796.
73. McAleer CW, Smith AST, Najjar S, Pirozzi K, Long CJ, Hickman JJ. Mechanistic investigation of adult myotube response to exercise and drug treatment in vitro using a multiplexed functional assay system. J Appl Physiol. 2014 Dec 1;117(11):1398–1405.
74. Mack DL, Guan X, Wagoner A, Walker SJ, Childers MK. Disease-in-a-dish: the contribution of patient-specific induced pluripotent stem cell technology to regenerative rehabilitation. Am J Phys Med Rehabil. 2014 Nov;93(11 Suppl 3):S155–68.
75. Braun R, Wang Z, Mack DL, Childers MK. Gene therapy for inherited muscle diseases: where genetics meets rehabilitation medicine. Am J Phys Med Rehabil. 2014 Nov;93(11 Suppl 3):S97–107.
76. Smith AST, Long CJ, McAleer C, Bobbitt N, Srinivasan B, Hickman JJ. Utilization of microscale silicon cantilevers to assess cellular contractile function in vitro. J Vis Exp. 2014 Oct 3;(92):e51866.
77. Smith AST, Long CJ, Pirozzi K, Najjar S, McAleer C, Vandenburgh HH, et al. A multiplexed chip-based assay system for investigating the functional development of human skeletal myotubes in vitro. J Biotechnol. 2014 Sep 20;185:15–18.
78. Wang Y, Sakano H, Beebe K, Brown MR, de Laat R, Bothwell M, et al. Intense and specialized dendritic localization of the fragile X mental retardation protein in binaural brainstem neurons: a comparative study in the alligator, chicken, gerbil, and human. J Comp Neurol. 2014 Jun 15;522(9):2107–2128.
79. Esch MB, Smith AST, Prot J-M, Oleaga C, Hickman JJ, Shuler ML. How multi-organ microdevices can help foster drug development. Adv Drug Deliv Rev. 2014 Apr;69-70:158–169.
80. Guan X, Mack DL, Moreno CM, Strande JL, Mathieu J, Shi Y, et al. Dystrophin-deficient cardiomyocytes derived from human urine: new biologic reagents for drug discovery. Stem Cell Res. 2014 Mar;12(2):467–480.
81. Childers MK, Joubert R, Poulard K, Moal C, Grange RW, Doering JA, et al. Gene therapy prolongs survival and restores function in murine and canine models of myotubular myopathy. Sci Transl Med. 2014 Jan 22;6(220):220ra10.
82. Smith AST, Long CJ, Berry BJ, McAleer C, Stancescu M, Molnar P, et al. Microphysiological systems and low-cost microfluidic platform with analytics. Stem Cell Res Ther. 2013 Dec 20;4 Suppl 1:S9.
83. Pirozzi KL, Long CJ, McAleer CW, Smith AST, Hickman JJ. Correlation of embryonic skeletal muscle myotube physical characteristics with contractile force generation on an atomic force microscope-based bio-microelectromechanical systems device. Appl Phys Lett. 2013 Aug 19;103(8):83108.
84. Padilla-Nash HM, McNeil NE, Yi M, Nguyen Q-T, Hu Y, Wangsa D, et al. Aneuploidy, oncogene amplification and epithelial to mesenchymal transition define spontaneous transformation of murine epithelial cells. Carcinogenesis. 2013 Aug;34(8):1929–1939.
85. Mirmalek-Sani S-H, Orlando G, McQuilling JP, Pareta R, Mack DL, Salvatori M, et al. Porcine pancreas extracellular matrix as a platform for endocrine pancreas bioengineering. Biomaterials. 2013 Jul;34(22):5488–5495.
86. Boulanger CA, Bruno RD, Mack DL, Gonzales M, Castro NP, Salomon DS, et al. Embryonic stem cells are redirected to non-tumorigenic epithelial cell fate by interaction with the mammary microenvironment. PLoS One. 2013 Apr 26;8(4):e62019.
87. Zhou Y, Mack DL, Williams JK, Mirmalek-Sani S-H, Moorefield E, Chun S-Y, et al. Genetic modification of primate amniotic fluid-derived stem cells produces pancreatic progenitor cells in vitro. Cells Tissues Organs (Print). 2013 Jan 8;197(4):269–282.
88. Smith AST, Long CJ, Pirozzi K, Hickman JJ. A functional system for high-content screening of neuromuscular junctions in vitro. Technology (Singap World Sci). 2013;1(1):37–48.
89. Schecterson LC, Sanchez JT, Rubel EW, Bothwell M. TrkB downregulation is required for dendrite retraction in developing neurons of chicken nucleus magnocellularis. J Neurosci. 2012 Oct 3;32(40):14000–14009.
90. Smith AST, Passey S, Greensmith L, Mudera V, Lewis MP. Characterization and optimization of a simple, repeatable system for the long term in vitro culture of aligned myotubes in 3D. J Cell Biochem. 2012 Mar;113(3):1044–1053.
91. Mudera V, Smith AST, Brady MA, Lewis MP. The effect of cell density on the maturation and contractile ability of muscle derived cells in a 3D tissue-engineered skeletal muscle model and determination of the cellular and mechanical stimuli required for the synthesis of a postural phenotype. J Cell Physiol. 2010 Nov;225(3):646–653.
92. Schecterson LC, Bothwell M. Neurotrophin receptors: Old friends with new partners. Dev Neurobiol. 2010 Apr;70(5):332–338.
93. Schecterson LC, Hudson MP, Ko M, Philippidou P, Akmentin W, Wiley J, et al. Trk activation in the secretory pathway promotes Golgi fragmentation. Mol Cell Neurosci. 2010 Apr;43(4):403–413.
94. Wiley JC, Meabon JS, Frankowski H, Smith EA, Schecterson LC, Bothwell M, et al. Phenylbutyric acid rescues endoplasmic reticulum stress-induced suppression of APP proteolysis and prevents apoptosis in neuronal cells. PLoS One. 2010 Feb 9;5(2):e9135.
95. Mandai K, Guo T, St Hillaire C, Meabon JS, Kanning KC, Bothwell M, et al. LIG family receptor tyrosine kinase-associated proteins modulate growth factor signals during neural development. Neuron. 2009 Sep 10;63(5):614–627.
96. Vilar M, Charalampopoulos I, Kenchappa RS, Simi A, Karaca E, Reversi A, et al. Activation of the p75 neurotrophin receptor through conformational rearrangement of disulphide-linked receptor dimers. Neuron. 2009 Apr 16;62(1):72–83.
97. Schecterson LC, Bothwell M. An all-purpose tool for axon guidance. Sci Signal. 2008 Nov 25;1(47):pe50.
98. Booth BW, Mack DL, Androutsellis-Theotokis A, McKay RDG, Boulanger CA, Smith GH. The mammary microenvironment alters the differentiation repertoire of neural stem cells. Proc Natl Acad Sci USA. 2008 Sep 30;105(39):14891–14896.
99. Strizzi L, Mancino M, Bianco C, Raafat A, Gonzales M, Booth BW, et al. Netrin-1 can affect morphogenesis and differentiation of the mouse mammary gland. J Cell Physiol. 2008 Sep;216(3):824–834.
100. Wiley JC, Smith EA, Hudson MP, Ladiges WC, Bothwell M. Fe65 stimulates proteolytic liberation of the beta-amyloid precursor protein intracellular domain. J Biol Chem. 2007 Nov 16;282(46):33313–33325.
101. Tesar PJ, Chenoweth JG, Brook FA, Davies TJ, Evans EP, Mack DL, et al. New cell lines from mouse epiblast share defining features with human embryonic stem cells. Nature. 2007 Jul 12;448(7150):196–199.
102. Mack DL, Boulanger CA, Callahan R, Smith GH. Expression of truncated Int6/eIF3e in mammary alveolar epithelium leads to persistent hyperplasia and tumorigenesis. Breast Cancer Res. 2007;9(4):R42.
103. Hatch NE, Hudson M, Seto ML, Cunningham ML, Bothwell M. Intracellular retention, degradation, and signaling of glycosylation-deficient FGFR2 and craniosynostosis syndrome-associated FGFR2C278F. J Biol Chem. 2006 Sep 15;281(37):27292–27305.
104. Bothwell M. Evolution of the neurotrophin signaling system in invertebrates. Brain Behav Evol. 2006 Aug 14;68(3):124–132.
105. Wiley JC, Hudson M, Kanning KC, Schecterson LC, Bothwell M. Familial Alzheimer’s disease mutations inhibit gamma-secretase-mediated liberation of beta-amyloid precursor protein carboxy-terminal fragment. J Neurochem. 2005 Sep;94(5):1189–1201.
106. Hatch NE, Nociti F, Swanson E, Bothwell M, Somerman M. FGF2 alters expression of the pyrophosphate/phosphate regulating proteins, PC-1, ANK and TNAP, in the calvarial osteoblastic cell line, MC3T3E1(C4). Connect Tissue Res. 2005;46(4-5):184–192.
107. Kanning KC, Hudson M, Amieux PS, Wiley JC, Bothwell M, Schecterson LC. Proteolytic processing of the p75 neurotrophin receptor and two homologs generates C-terminal fragments with signaling capability. J Neurosci. 2003 Jul 2;23(13):5425–5436.