Cortical neurons derived from human pluripotent stem cells lacking FMRP display altered spontaneous firing patterns.
Open accessAbstract
Background: Fragile X syndrome (FXS), a neurodevelopmental disorder, is a leading monogenetic cause of intellectual disability and autism spectrum disorder. Notwithstanding the extensive studies using rodent and other pre-clinical models of FXS, which have provided detailed mechanistic insights into the pathophysiology of this disorder, it is only relatively recently that human stem cell-derived neurons have been employed as a model system to further our understanding of the pathophysiological events that may underlie FXS. Our study assesses the physiological properties of human pluripotent stem cell-derived cortical neurons lacking fragile X mental retardation protein (FMRP). Methods: Electrophysiological whole-cell voltage- and current-clamp recordings were performed on two control and three FXS patient lines of human cortical neurons derived from induced pluripotent stem cells. In addition, we also describe the properties of an isogenic pair of lines in one of which FMR1 gene expression has been silenced. Results: Neurons lacking FMRP displayed bursts of spontaneous action potential firing that were more frequent but shorter in duration compared to those recorded from neurons expressing FMRP. Inhibition of large conductance Ca 2+-activated K+ currents and the persistent Na+ current in control neurons phenocopies action potential bursting observed in neurons lacking FMRP, while in neurons lacking FMRP pharmacological potentiation of voltage-dependent Na+ channels phenocopies action potential bursting observed in control neurons. Notwithstanding the changes in spontaneous action potential firing, we did not observe any differences in the intrinsic properties of neurons in any of the lines examined. Moreover, we did not detect any differences in the properties of miniature excitatory postsynaptic currents in any of the lines. Conclusions: Pharmacological manipulations can alter the action potential burst profiles in both control and FMRP-null human cortical neurons, making them appear like their genetic counterpart. Our studies indicate that FMRP targets that have been found in rodent models of FXS are also potential targets in a human-based model system, and we suggest potential mechanisms by which activity is altered.
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Blocking antibody to S100B induces disordered firing in co-cultures of control neurons and control astrocytes, demonstrating that astrocyte secreted factors can influence firing patterns of glutamatergic neurons.
“Furthermore, blocking antibody to S100B induced disordered firing in co-cultures of control neurons and control astrocytes (Das Sharma et al., 2023), demonstrating that astrocyte secreted factors can influence the firing patterns of glutamatergic neurons (Das Sharma et al., 2020).”
From Astrocytes in fragile X syndrome (2024) · cites this paper as Das Sharma et al., 2020
The 2020 citation is attached to the conclusion about astrocyte-secreted factors, not the S100B antibody experiment cited to 2023. The Results section reports firing differences in neurons co-cultured with wild-type mouse astrocytes and describes those differences as cell-autonomous; it does not test whether astrocyte-secreted factors influence firing. Searches for S100B and secreted factors found no passage addressing that conclusion. (LLM, conf=0.75)
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Cites
FMRP Stalls Ribosomal Translocation on mRNAs Linked to Synaptic Function and Autism
Darnell, J. C., Van Driesche, S. J., Zhang, C., Hung, K. Y. S., Mele, A., Fraser, C. E., Stone, E. F., Chen, C., Fak, J. J., Chi, S. W., et al. (2011) · Cell · doi:10.1016/j.cell.2011.06.013 · Cited by 2,072 · see citing works
FMR1 and the fragile X syndrome: Human genome epidemiology review
Crawford, D. C., Acuña, J. M., & Sherman, S. L. (2001) · Genetics in Medicine · doi:10.1097/00125817-200109000-00006 · Cited by 711 · see citing works
Gibson, J. R., Bartley, A. F., Hays, S. A., & Huber, K. M. (2008) · Journal of Neurophysiology · doi:10.1152/jn.90752.2008 · Cited by 529 · see citing works
Altered Neuronal and Circuit Excitability in Fragile X Syndrome
Contractor, A., Klyachko, V. A., & Portera‐Cailliau, C. (2015) · Neuron · doi:10.1016/j.neuron.2015.06.017 · Cited by 425 · see citing works
Deng, P.-Y., Rotman, Z., Blundon, J. A., Cho, Y., Cui, J., Cavalli, V., Zakharenko, S. S., & Klyachko, V. A. (2013) · Neuron · doi:10.1016/j.neuron.2012.12.018 · Cited by 354 · see citing works
The fragile X prevalence paradox
Hagerman, P. J. (2008) · Journal of Medical Genetics · doi:10.1136/jmg.2008.059055 · Cited by 343 · see citing works
Johnson, M. A., Weick, J. P., Pearce, R. A., & Zhang, S.-C. (2007) · The Journal of Neuroscience · doi:10.1523/jneurosci.4562-06.2007 · Cited by 318 · see citing works
Mechanisms and consequences of action potential burst firing in rat neocortical pyramidal neurons
Williams, S. R., & Stuart, G. J. (1999) · The Journal of Physiology · doi:10.1111/j.1469-7793.1999.00467.x · Cited by 298 · see citing works
Eiges, R., Urbach, A., Malcov, M., Frumkin, T., Schwartz, T., Amit, A., Yaron, Y., Eden, A., Yanuka, O., Benvenisty, N., et al. (2007) · Cell stem cell · doi:10.1016/j.stem.2007.09.001 · Cited by 287 · see citing works
Circuit level defects in the developing neocortex of fragile X mice
Gonçalves, J. T., Anstey, J. E., Golshani, P., & Portera-Cailliau, C. (2013) · Nature Neuroscience · doi:10.1038/nn.3415 · Cited by 279 · see citing works
Toward Fulfilling the Promise of Molecular Medicine in Fragile X Syndrome
Krueger, D. D., & Bear, M. F. (2011) · Annual Review of Medicine · doi:10.1146/annurev-med-061109-134644 · Cited by 259 · see citing works
Ionic Mechanisms Underlying Repetitive High-Frequency Burst Firing in Supragranular Cortical Neurons
Brumberg, J. C., Nowak, L. G., & McCormick, D. A. (2000) · Journal of Neuroscience · doi:10.1523/jneurosci.20-13-04829.2000 · Cited by 234 · see citing works
Odawara, A., Katoh, H., Matsuda, N., & Suzuki, I. (2016) · Scientific Reports · doi:10.1038/srep26181 · Cited by 229 · see citing works
Dendritic channelopathies contribute to neocortical and sensory hyperexcitability in Fmr1−/y mice
Zhang, Y., Bonnan, A., Bony, G., Férézou, I., Pietropaolo, S., Ginger, M. R., Sans, N., Rossier, J., Oostra, B. A., LeMasson, G., et al. (2014) · Nature Neuroscience · doi:10.1038/nn.3864 · Cited by 227 · see citing works
Selvaraj, B. T., Livesey, M. R., Zhao, C., Gregory, J. M., James, O. T., Cleary, E. M., Chouhan, A. K., Gane, A. B., Perkins, E., Dando, O., et al. (2018) · Nature Communications · doi:10.1038/s41467-017-02729-0 · Cited by 200 · see citing works
Self-citationAltered differentiation of neural stem cells in fragile X syndrome
Castrén, M., Tervonen, T., Kärkkäinen, V., Heinonen, S., Castrén, E., Larsson, K., Bakker, C. E., Oostra, B. A., & Åkerman, K. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0508995102 · Cited by 176 · see citing works
Astrocytes prevent abnormal neuronal development in the fragile X mouse.
Jacobs, S., & Doering, L. C. (2010) · The Journal of Neuroscience · doi:10.1523/jneurosci.5027-09.2010 · Cited by 170 · see citing works
Hagerman, R. J., Amiri, K. M. A., & Cronister, A. (1991) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320380223 · Cited by 165 · see citing works
Gross, C., Yao, X., Pong, D. L., Jeromin, A., & Bassell, G. J. (2011) · The Journal of Neuroscience · doi:10.1523/jneurosci.6661-10.2011 · Cited by 161 · see citing works
Self-citationIn vitro post-meiotic germ cell development from human embryonic stem cells
Aflatoonian, B., Ruban, L., Jones, M., Aflatoonian, R., Fazeli, A., & Moore, H. D. M. (2009) · Human Reproduction · doi:10.1093/humrep/dep334 · Cited by 149 · see citing works
Zalfa, F., Adinolfi, S., Napoli, I., Kühn‐Hölsken, E., Urlaub, H., Achsel, T., Pastore, A., & Bagni, C. (2005) · Journal of Biological Chemistry · doi:10.1074/jbc.m504286200 · Cited by 139 · see citing works
Behavioral phenotype of fragile X syndrome: DSM‐III‐R autistic behavior in male children
Reiss, A. L., & Freund, L. S. (1992) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320430106 · Cited by 138 · see citing works
Strumbos, J. G., Brown, M. R., Kronengold, J., Polley, D. B., & Kaczmarek, L. K. (2010) · The Journal of Neuroscience · doi:10.1523/jneurosci.1125-10.2010 · Cited by 135 · see citing works
Parri, H. R., & Crunelli, V. (1998) · Journal of Neuroscience · doi:10.1523/jneurosci.18-03-00854.1998 · Cited by 124 · see citing works
Timing of the absence of FMR1 expression in full mutation chorionic villi
Willemsen, R., Bontekoe, C. J. M., Severijnen, L.-A. W. F. M., & Oostra, B. A. (2002) · Human Genetics · doi:10.1007/s00439-002-0723-5 · Cited by 114 · see citing works
Franceschetti, S., Guatteo, E., Panzica, F., Sancini, G., Wanke, E., & AVANZINI, G. (1995) · Brain Research · doi:10.1016/0006-8993(95)00807-3 · Cited by 103 · see citing works
Telias, M., Segal, M., & Ben‐Yosef, D. (2012) · Developmental Biology · doi:10.1016/j.ydbio.2012.11.031 · Cited by 103 · see citing works
Early Postnatal Plasticity in Neocortex of <i>Fmr1</i> Knockout Mice
Desai, N. S., Casimiro, T. M., Gruber, S. M., & Vanderklish, P. W. (2006) · Journal of Neurophysiology · doi:10.1152/jn.00221.2006 · Cited by 92 · see citing works
Deng, P., & Klyachko, V. A. (2016) · Cell Reports · doi:10.1016/j.celrep.2016.08.046 · Cited by 79 · see citing works
Fragile X mental retardation protein controls ion channel expression and activity
Ferron, L. (2016) · The Journal of Physiology · doi:10.1113/jp270675 · Cited by 76 · see citing works
Deng, P., & Klyachko, V. A. (2015) · The Journal of Physiology · doi:10.1113/jp271031 · Cited by 73 · see citing works
Altered dendritic spine function and integration in a mouse model of fragile X syndrome
Booker, S. A., Domanski, A. P. F., Dando, O., Jackson, A. D., Isaac, J. T. R., Hardingham, G. E., Wyllie, D. J. A., & Kind, P. C. (2019) · Nature Communications · doi:10.1038/s41467-019-11891-6 · Cited by 70 · see citing works
Self-citationFunctional Deficiencies in Fragile X Neurons Derived from Human Embryonic Stem Cells
Telias, M., Kuznitsov-Yanovsky, L., Segal, M., & Ben‐Yosef, D. (2015) · Journal of Neuroscience · doi:10.1523/jneurosci.0317-15.2015 · Cited by 68 · see citing works
Normal Neurogenesis but Abnormal Gene Expression in Human Fragile X Cortical Progenitor Cells
Bhattacharyya, A., McMillan, E., Wallace, K., Tubon, T. C., Capowski, E. E., & Svendsen, C. N. (2008) · Stem Cells and Development · doi:10.1089/scd.2007.0073 · Cited by 61 · see citing works
Self-citationDomanski, A. P. F., Booker, S. A., Wyllie, D. J. A., Isaac, J. T. R., & Kind, P. C. (2019) · Nature Communications · doi:10.1038/s41467-019-12736-y · Cited by 56 · see citing works
Self-citationHomeostatic Intrinsic Plasticity Is Functionally Altered in Fmr1 KO Cortical Neurons
Bülow, P., Murphy, T. J., Bassell, G. J., & Wenner, P. (2019) · Cell Reports · doi:10.1016/j.celrep.2019.01.035 · Cited by 53 · see citing works
Self-citationRouth, B. N., Rathour, R. K., Baumgardner, M. E., Kalmbach, B. E., Johnston, D., & Brager, D. H. (2017) · The Journal of Physiology · doi:10.1113/jp274258 · Cited by 51 · see citing works
Bilican, B., Livesey, M. R., Haghi, G., Qiu, J., Burr, K., Siller, R., Hardingham, G. E., Wyllie, D. J. A., & Chandran, S. (2014) · PLoS ONE · doi:10.1371/journal.pone.0085932 · Cited by 47 · see citing works
Self-citationMagistretti, J., & Alonso, A. (2002) · The Journal of General Physiology · doi:10.1085/jgp.20028676 · Cited by 43 · see citing works
Modeling Neurodevelopmental Disorders Using Human Pluripotent Stem Cells
Telias, M., & Ben-Yosef, D. (2014) · Stem Cell Reviews and Reports · doi:10.1007/s12015-014-9507-2 · Cited by 42 · see citing works
Partial FMRP expression is sufficient to normalize neuronal hyperactivity in Fragile X neurons
Graef, J. D., Wu, H., Ng, C., Sun, C., Villegas, V., Qadir, D., Jesseman, K., Warren, S. T., Jaenisch, R., Cacace, A. M., et al. (2019) · European Journal of Neuroscience · doi:10.1111/ejn.14660 · Cited by 42 · see citing works
Sensory hypo-excitability in a rat model of fetal development in Fragile X Syndrome
Berzhanskaya, J., Phillips, M. A., Shen, J., & Colonnese, M. T. (2016) · Scientific Reports · doi:10.1038/srep30769 · Cited by 41 · see citing works
Matsuda, N., Odawara, A., Katoh, H., Okuyama, N., Yokoi, R., & Suzuki, I. (2018) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2018.02.117 · Cited by 40 · see citing works
Immature Responses to GABA in Fragile X Neurons Derived from Human Embryonic Stem Cells
Telias, M., Segal, M., & Ben‐Yosef, D. (2016) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2016.00121 · Cited by 35 · see citing works
FMRP Interacts with C/D Box snoRNA in the Nucleus and Regulates Ribosomal RNA Methylation
D’Souza, M. N., Gowda, N. K. C., Tiwari, V., Babu, R. O., Anand, P., Dastidar, S. G., Singh, R., James, O. G., Selvaraj, B. T., Pal, R., et al. (2018) · iScience · doi:10.1016/j.isci.2018.11.007 · Cited by 34 · see citing works
Self-citationMaturation of AMPAR Composition and the GABA A R Reversal Potential in hPSC-Derived Cortical Neurons
Livesey, M. R., Bilican, B., Qiu, J., Rzechorzek, N. M., Haghi, G., Burr, K., Hardingham, G. E., Chandran, S., & Wyllie, D. J. A. (2014) · Journal of Neuroscience · doi:10.1523/jneurosci.5410-13.2014 · Cited by 33 · see citing works
Self-citationHuman pluripotent stem cell models of Fragile X syndrome
Bhattacharyya, A., & Zhao, X. (2015) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2015.11.011 · Cited by 33 · see citing works
Excitability is increased in hippocampal CA1 pyramidal cells of Fmr1 knockout mice
Luque, M. A., Beltrán‐Matas, P., Marin, M. C., Torres, B., & Herrero, L. (2017) · PLoS ONE · doi:10.1371/journal.pone.0185067 · Cited by 32 · see citing works
Yamada-Hanff, J., & Bean, B. P. (2015) · Journal of Neurophysiology · doi:10.1152/jn.00489.2015 · Cited by 31 · see citing works
Reevaluation of FMR1 Hypermethylation Timing in Fragile X Syndrome
Mor‐Shaked, H., & Eiges, R. (2018) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2018.00031 · Cited by 27 · see citing works
Livesey, M. R., Magnani, D., Hardingham, G. E., Chandran, S., & Wyllie, D. J. A. (2015) · The Journal of Physiology · doi:10.1113/jp270660 · Cited by 24 · see citing works
Self-citationJames, O. T., Livesey, M. R., Qiu, J., Dando, O., Bilican, B., Haghi, G., Rajan, R., Burr, K., Hardingham, G. E., Chandran, S., et al. (2014) · The Journal of Physiology · doi:10.1113/jphysiol.2014.278994 · Cited by 19 · see citing works
Self-citationZhang, L., Liang, Z., Zhu, P., Li, M., Yi, Y., Liao, W., & Su, T. (2016) · Experimental Neurology · doi:10.1016/j.expneurol.2016.03.025 · Cited by 14 · see citing works
Single-Cell and Neuronal Network Alterations in an In Vitro Model of Fragile X Syndrome
Moskalyuk, A., Vijver, S. V. D., Verstraelen, P., Vos, W. H. D., Kooy, R. F., & Giugliano, M. (2019) · Cerebral Cortex · doi:10.1093/cercor/bhz068 · Cited by 12 · see citing works
Miller (1999) · doi:10.1002/(sici)1096-8628(19990402)83:4
Flagged first, then by citations
Research works printed but not linked: 1
- [5]Hagerman RJ Hills J Scharfenaker S Lewis H Fragile X syndrome and selective mutism Am J Med Genet 1999 83 4 313 317 10208168
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Astrocytes mediate cell non-autonomous correction of aberrant firing in human FXS neurons.
Sharma, S. D., Reddy, B. K., Pal, R., Ritakari, T. E., Cooper, J. D., Selvaraj, B. T., Kind, P. C., Chandran, S., Wyllie, D. J. A., & Chattarji, S. (2023) · Cell Reports · doi:10.1016/j.celrep.2023.112344 · Cited by 31 · see citing works
Self-citationCourchesne, V., Bedford, R., Pickles, A. R., Duku, E., Kerns, C. M., Mirenda, P., Bennett, T. A., Georgiades, S., Smith, I. M., Ungar, W. J., et al. (2021) · Molecular Autism · doi:10.1186/s13229-021-00461-7 · Cited by 27 · see citing works
FMRP Sustains Presynaptic Function via Control of Activity-Dependent Bulk Endocytosis
Bonnycastle, K., Kind, P. C., & Cousin, M. A. (2022) · Journal of Neuroscience · doi:10.1523/jneurosci.0852-21.2021 · Cited by 21 · see citing works
Self-citationWinden, K. D., Pham, T. T., Teaney, N. A., Ruíz, J. P., Chen, R., Chen, C.-D., & Şahin, M. (2023) · Cell Reports · doi:10.1016/j.celrep.2023.112838 · Cited by 18 · see citing works
Bursting dynamics in a spiking neuron with a memristive voltage-gated channel
Wu, J., Wang, K., Schneegans, O., Stoliar, P., & Rozenberg, M. J. (2023) · Neuromorphic Computing and Engineering · doi:10.1088/2634-4386/ad139b · Cited by 16 · see citing works
Impaired Functional Connectivity Underlies Fragile X Syndrome
Gildin, L., Rauti, R., Vardi, O., Kuznitsov-Yanovsky, L., Maoz, B. M., Segal, M., & Ben‐Yosef, D. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23042048 · Cited by 11 · see citing works
Across Dimensions: Developing 2D and 3D Human iPSC-Based Models of Fragile X Syndrome
Lee, A., Xu, J., Wen, Z., & Jin, P. (2022) · Cells · doi:10.3390/cells11111725 · Cited by 10 · see citing works
Astrocytes in fragile X syndrome
Talvio, K., & Castrén, M. L. (2024) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2023.1322541 · Cited by 8 · see citing works
From wings to whiskers to stem cells: why every model matters in fragile X syndrome research
Sandoval, S. O., Méndez-Albelo, N. M., Xu, Z., & Zhao, X. (2024) · Journal of Neurodevelopmental Disorders · doi:10.1186/s11689-024-09545-w · Cited by 8 · see citing works
Ramesh, V., Tsoukala, E., Kougianou, I., Kozić, Z., Burr, K., Viswanath, B., Hampton, D. W., Story, D., Reddy, B. K., Pal, R., et al. (2025) · Glia · doi:10.1002/glia.24680 · Cited by 7 · see citing works
Self-citationAn in-depth review of the function of RNA-binding protein FXR1 in neurodevelopment
Méndez-Albelo, N. M., Sandoval, S. O., Xu, Z., & Zhao, X. (2024) · Cell and Tissue Research · doi:10.1007/s00441-024-03912-8 · Cited by 7 · see citing works
Age-Dependent Dysregulation of APP in Neuronal and Skin Cells from Fragile X Individuals
Cencelli, G., Pacini, L., Luca, A. D., Messia, I., Gentile, A., Kang, Y., Nobile, V., Tabolacci, E., Jin, P., Farace, M. G., et al. (2023) · Cells · doi:10.3390/cells12050758 · Cited by 6 · see citing works
Bonnycastle, K., Nawaz, M. S., Kind, P. C., & Cousin, M. A. (2025) · Molecular Autism · doi:10.1186/s13229-025-00660-6 · Cited by 5 · see citing works
Self-citationSubrahmanyam, R., Dwivedi, D., Rashid, Z., Bonnycastle, K., Cousin, M. A., & Chattarji, S. (2021) · Journal of Neurochemistry · doi:10.1111/jnc.15484 · Cited by 5 · see citing works
Self-citationTalvio, K., Minkeviciene, R., Townsley, K. G., Achuta, V. S., Huckins, L. M., Corcoran, P., Brennand, K. J., & Castrén, M. L. (2022) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2022.1034679 · Cited by 5 · see citing works
Delayed recruitment of activity‐dependent bulk endocytosis in Fmr1 knockout neurons
Kim, N., Bonnycastle, K., Kind, P. C., & Cousin, M. A. (2024) · Journal of Neurochemistry · doi:10.1111/jnc.16178 · Cited by 4 · see citing works
Self-citationYang, Z., Teaney, N. A., Buttermore, E. D., Şahin, M., & Saber, W. A. (2025) · Frontiers in Neuroscience · doi:10.3389/fnins.2024.1524577 · Cited by 4 · see citing works
Geng, J., Wang, X., Pan, J., Khan, D., Pimcharoen, S., Zhang, Y., Mosammaparast, N., Hirose, S., Petrucelli, L., Brandman, O., et al. (2025) · Science Translational Medicine · doi:10.1126/scitranslmed.adq3551 · Cited by 4 · see citing works
A Dynamic Spike Sorter for Multiscale Nanoelectrode Array Recordings
Shukla, S., Walsh, C., Bansal, S., Leugering, J., Uppal, A., Jaltare, V., Cauwenberghs, G., Chalasani, S. H., & Jahed, Z. (2025) · Advanced Materials Interfaces · doi:10.1002/admi.202500158 · Cited by 2 · see citing works
Dey, S., & Das, A. (2026) · Brain Research · doi:10.1016/j.brainres.2026.150164 · Cited by 2 · see citing works
Fink, J. J., Delaney-Busch, N., Dawes, R. P., Nanou, E., Folts, C. J., Harikrishnan, K., Hempel, C. M., Upadhyay, H., Nguyen, T. M., Shroff, H., et al. (2024) · Communications Biology · doi:10.1038/s42003-024-07120-6 · Cited by 2 · see citing works
Pham, K., Malachowski, T., Zhou, L., Kim, J. H., Chuanbin, S., & Phillips‐Cremins, J. E. (2025) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2025.03.19.644148 · Cited by 2 · see citing works
Hu, Z., Yan, Q., Wan, J., Fan, Y., & Liu, J. (2025) · Movement Disorders · doi:10.1002/mds.70081 · Cited by 1 · see citing works
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Ren, X., Sirois, C. L., Doudlah, R. C., Dayley, E. E., Méndez-Albelo, N. M., Hai, A., Rosenberg, A., & Zhao, X. (2026) · Stem Cell Reports · doi:10.1016/j.stemcr.2026.102872 · Cited by 1 · see citing works
Developing clinically translatable screens using iPSC-derived neural cells
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- Authors
- Das Sharma, S., Pal, R., Reddy, B. K., Selvaraj, B. T., Raj, N., Samaga, K. K., Srinivasan, D. J., Ornelas, L., Sareen, D., Livesey, M. R., Bassell, G. J., Svendsen, C. N., Kind, P. C., Chandran, S., Chattarji, S., & Wyllie, D. J. A.
- Affiliations
- Institute for Stem Cell Biology and Regenerative Medicine (IN)
- UK Dementia Research Institute (GB)
- Emory University (US)
- University of Trans-Disciplinary Health Sciences and Technology (IN)
- Cedars-Sinai Medical Center (US)
- University of Edinburgh (GB)
- Tata Institute of Fundamental Research (IN)
- Corresponding author
- Chattarji, Sumantra
- Wyllie, David J. A.
- ORCID
- Venue
- Molecular Autism
- Published
- 2020-06-19
- Volume
- 11
- Issue
- 1
- Pages
- 52-52
- ISSN
- 2040-2392 (ISSN-L)
- PMC
- PMC7304215
- OpenAlex
- W3036227905
- Cited by
- 28
- References
- 55
- Citations by year
- 2026: 2 · 2025: 7 · 2024: 6 · 2023: 6 · 2022: 5 · 2021: 2
- FWCI
- 1.91
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- Molecular Autism (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Edinburgh Research Explorer (University of Edinburgh) (submittedVersion, other-oa)
- Funders
- Department of Biotechnology, Government of India
- Simons Foundation Autism Research Initiative (529085)
- Patrick Wild Centre
- National Institutes of Health (NIH1U54HD082013)
- Cedars-Sinai Programmatic Funds
- David and Janet Polak Foundation
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Neuroscience and Neuropharmacology Research
- Life Sciences › Neuroscience › Cognitive Neuroscience › Neural dynamics and brain function
- Keywords
- fragile X syndrome, FMRP, human pluripotent stem cell-derived neurons, cortical neurons, induced pluripotent stem cells, action potential bursting, miniature excitatory postsynaptic currents
- MeSH terms
- Action Potentials, Adolescent, Animals, Cell Differentiation, Cerebral Cortex, Child, Preschool, Humans, Indoles, Male, Neurons, Veratridine, Sodium Channels, Riluzole, Mice, Fragile X Mental Retardation Protein, Fragile X Messenger Ribonucleoprotein 1, Young Adult, Induced Pluripotent Stem Cells
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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