Fragile X mental retardation protein is required for rapid experience-dependent regulation of the potassium channel Kv3.1b
Open accessAbstract
Fragile X mental retardation protein (FMRP) is an RNA-binding protein that regulates synaptic plasticity by repressing translation of specific mRNAs. We found that FMRP binds mRNA encoding the voltage-gated potassium channel Kv3.1b in brainstem synaptosomes. To explore the regulation of Kv3.1b by FMRP, we investigated Kv3.1b immunoreactivity and potassium currents in the auditory brainstem sound localization circuit of male mice. The unique features of this circuit allowed us to control neuronal activity in vivo by exposing animals to high-frequency, amplitude-modulated stimuli, which elicit predictable and stereotyped patterns of input to the anterior ventral cochlear nucleus (AVCN) and medial nucleus of the trapezoid body (MNTB). In wild-type (WT) animals, Kv3.1b is expressed along a tonotopic gradient in the MNTB, with highest levels in neurons at the medial, high-frequency end. At baseline,Fmr1−/−mice, which lack FMRP, displayed dramatically flattened tonotopicity in Kv3.1b immunoreactivity and K+currents relative to WT controls. Moreover, after 30 min of acoustic stimulation, levels of Kv3.1b immunoreactivity were significantly elevated in both the MNTB and AVCN of WT, but not Fmr1−/−, mice. These results suggest that FMRP is necessary for maintenance of the gradient in Kv3.1b protein levels across the tonotopic axis of the MNTB, and are consistent with a role for FMRP as a repressor of protein translation. Using numerical simulations, we demonstrate that Kv3.1b tonotopicity may be required for accurate encoding of stimulus features such as modulation rate, and that disruption of this gradient, as occurs in Fmr1−/−animals, degrades processing of this information.
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
FMRP is expressed throughout the auditory system, and the cortical habituation deficits may have multiple origins (Strumbos et al., 2010; Wang et al., 2014; Rotschafer et al., 2015).
“However, FMRP is expressed across the auditory system and the observed cortical habituation deficits may have multiple origins (Strumbos et al., 2010; Wang et al., 2014; Rotschafer et al., 2015).”
From Matrix metalloproteinase-9 deletion rescues auditory evoked potential habituation deficit in a mouse model of Fragile X Syndrome (2016) · cites this paper as Strumbos et al., 2010
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Cites
Brown, V., Jin, P., Ceman, S., Darnell, J. C., O'Donnell, W. T., Tenenbaum, S. A., Jin, X., Feng, Y., Wilkinson, K. D., Keene, J. D., et al. (2001) · Cell · doi:10.1016/s0092-8674(01)00568-2 · Cited by 1,109 · see citing works
Fragile X Syndrome: Loss of Local mRNA Regulation Alters Synaptic Development and Function
Bassell, G. J., & Warren, S. T. (2008) · Neuron · doi:10.1016/j.neuron.2008.10.004 · Cited by 1,051 · see citing works
On The Cellular and Network Bases of Epileptic Seizures
McCormick, D. A., & Contreras, D. (2001) · Annual Review of Physiology · doi:10.1146/annurev.physiol.63.1.815 · Cited by 1,006 · see citing works
Fragile X mental retardation protein targets G quartet mRNAs important for neuronal function
Darnell, J. C., Jensen, K. B., Jin, P., Brown, V., Warren, S. T., & Darnell, R. B. (2001) · Cell · doi:10.1016/s0092-8674(01)00566-9 · Cited by 952 · see citing works
Kv3 channels: voltage-gated K+ channels designed for high-frequency repetitive firing
Rudy, B., & McBain, C. J. (2001) · Trends in Neurosciences · doi:10.1016/s0166-2236(00)01892-0 · Cited by 781 · see citing works
FMR1 Protein: Conserved RNP Family Domains and Selective RNA Binding
Ashley, C. T., Wilkinson, K. D., Reines, D., & Warren, S. T. (1993) · Science · doi:10.1126/science.7692601 · Cited by 741 · see citing works
Zalfa, F., Giorgi, M., Primerano, B., Moro, A., Di Penta, A., Reis, S., Oostra, B., & Bagni, C. (2003) · Cell · doi:10.1016/s0092-8674(03)00079-5 · Cited by 676 · see citing works
High-frequency firing helps replenish the readily releasable pool of synaptic vesicles
Wang, L., & Kaczmarek, L. K. (1998) · Nature · doi:10.1038/28645 · Cited by 614 · see citing works
Self-citationEvidence that fragile X mental retardation protein is a negative regulator of translation
Laggerbauer, B. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.4.329 · Cited by 571 · see citing works
Taschenberger, H., & von Gersdorff, H. (2000) · Journal of Neuroscience · doi:10.1523/jneurosci.20-24-09162.2000 · Cited by 486 · see citing works
Guinan, J. J., Norris, B. E., & Guinan, S. S. (1972) · International Journal of Neuroscience · doi:10.3109/00207457209164756 · Cited by 474 · see citing works
The fragile X mental retardation protein inhibits translation via interacting with mRNA
Li, Z. (2001) · Nucleic Acids Research · doi:10.1093/nar/29.11.2276 · Cited by 448 · see citing works
Muddashetty, R. S., Kelić, S., Gross, C., Xu, M., & Bassell, G. J. (2007) · The Journal of Neuroscience · doi:10.1523/jneurosci.0937-07.2007 · Cited by 421 · see citing works
SYNAPTIC MECHANISMS FOR CODING TIMING IN AUDITORY NEURONS
Trussell, L. O. (1999) · Annual Review of Physiology · doi:10.1146/annurev.physiol.61.1.477 · Cited by 412 · see citing works
Forsythe, I. D. (1994) · The Journal of Physiology · doi:10.1113/jphysiol.1994.sp020303 · Cited by 360 · see citing works
Contribution of the Kv3.1 potassium channel to high‐frequency firing in mouse auditory neurones
Wang, L., Gan, L., Forsythe, I. D., & Kaczmarek, L. K. (1998) · The Journal of Physiology · doi:10.1111/j.1469-7793.1998.183bo.x · Cited by 357 · see citing works
Self-citationFragile X mice develop sensory hyperreactivity to auditory stimuli
Chen, L., & Toth, M. (2001) · Neuroscience · doi:10.1016/s0306-4522(01)00036-7 · Cited by 345 · see citing works
Lu, R., Wang, H., Liang, Z., Ku, L., O'Donnell, W. T., Li, W., Warren, S. T., & Feng, Y. (2004) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0404995101 · Cited by 322 · see citing works
Synaptic Mitochondria Are More Susceptible to Ca2+Overload than Nonsynaptic Mitochondria
Brown, M. R., Sullivan, P. G., & Geddes, J. W. (2006) · Journal of Biological Chemistry · doi:10.1074/jbc.m510303200 · Cited by 297 · see citing works
Self-citationFragile X mental retardation protein is associated with translating polyribosomes in neuronal cells
Stefani, G., Fraser, C. E., Darnell, J. C., & Darnell, R. B. (2004) · The Journal of Neuroscience · doi:10.1523/jneurosci.2306-04.2004 · Cited by 274 · see citing works
(1996) · doi:10.1093/oso/9780195096842.001.0001 · Cited by 251 · see citing works
Epilepsy and EEG Findings in Males with Fragile X Syndrome
Musumeci, S. A., Hagerman, R. J., Ferri, R., Bosco, P., Bernardina, B. D., Tassinari, C. A., Sarro, G. D., & Elia, M. (1999) · Epilepsia · doi:10.1111/j.1528-1157.1999.tb00824.x · Cited by 250 · see citing works
Barnes‐Davies, M., & Forsythe, I. D. (1995) · The Journal of Physiology · doi:10.1113/jphysiol.1995.sp020974 · Cited by 249 · see citing works
Weiser, M., Bueno, E., Sekirnjak, C., Martone, M. E., Baker, H., Hillman, D. E., Chen, S., Thornhill, W. B., Ellisman, M. H., & Rudy, B. (1995) · Journal of Neuroscience · doi:10.1523/jneurosci.15-06-04298.1995 · Cited by 241 · see citing works
Expression of the mRNAs for the Kv3.1 potassium channel gene in the adult and developing rat brain
Perney, T. M., Marshall, J., Martin, K. A., Hockfield, S. J., & Kaczmarek, L. K. (1992) · Journal of Neurophysiology · doi:10.1152/jn.1992.68.3.756 · Cited by 224 · see citing works
Self-citationCa2 channel‐regulated neuronal gene expression
Finkbeiner, S. M., & Greenberg, M. E. (1998) · Journal of Neurobiology · doi:10.1002/(sici)1097-4695(199810)37:1 · Cited by 202 · see citing works
Self-citationSommer, I. E. C., Lingenh�hl, K., & Friauf, E. (1993) · Experimental Brain Research · doi:10.1007/bf00229781 · Cited by 197 · see citing works
Distinct Roles of Kv1 and Kv3 Potassium Channels at the Calyx of Held Presynaptic Terminal
Ishikawa, T., Nakamura, Y., Saitoh, N., Li, W., Iwasaki, S., & Takahashi, T. (2003) · Journal of Neuroscience · doi:10.1523/jneurosci.23-32-10445.2003 · Cited by 181 · see citing works
Envelope Coding in the Lateral Superior Olive. III. Comparison With Afferent Pathways
Joris, P. X., & Yin, T. C. T. (1998) · Journal of Neurophysiology · doi:10.1152/jn.1998.79.1.253 · Cited by 153 · see citing works
Localization of two high‐threshold potassium channel subunits in the rat central auditory system
Li, W., Kaczmarek, L. K., & Perney, T. M. (2001) · The Journal of Comparative Neurology · doi:10.1002/cne.1279 · Cited by 143 · see citing works
Self-citationKv3.4 subunits enhance the repolarizing efficiency of Kv3.1 channels in fast-spiking neurons
Baranauskas, G., Tkatch, T., Nagata, K., Yeh, J. Z., & Surmeier, D. J. (2003) · Nature Neuroscience · doi:10.1038/nn1019 · Cited by 139 · see citing works
Macica, C. M., von Hehn, C. A. A., Wang, L., Ho, C. S., Yokoyama, S., Joho, R. H., & Kaczmarek, L. K. (2003) · Journal of Neuroscience · doi:10.1523/jneurosci.23-04-01133.2003 · Cited by 134 · see citing works
Self-citationFragile X-related proteins regulate mammalian circadian behavioral rhythms
Zhang, J., Fang, Z., Jud, C., Vansteensel, M. J., Kaasik, K., Lee, C. C., Albrecht, U., Tamanini, F., Meijer, J. H., Oostra, B. A., et al. (2008) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2008.06.003 · Cited by 131 · see citing works
Kopp‐Scheinpflug, C., Lippe, W. R., Dörrscheidt, G. J., & R�bsamen, R. (2003) · Journal of the Association for Research in Otolaryngology · doi:10.1007/s10162-002-2010-5 · Cited by 129 · see citing works
Fast delayed rectifier potassium current is required for circadian neural activity
Itri, J. N., Michel, S. H., Vansteensel, M. J., Meijer, J. H., & Colwell, C. S. (2005) · Nature Neuroscience · doi:10.1038/nn1448 · Cited by 129 · see citing works
Song, P., Yang, Y., Barnes‐Davies, M., Bhattacharjee, A., Hamann, M., Forsythe, I. D., Oliver, D. L., & Kaczmarek, L. K. (2005) · Nature Neuroscience · doi:10.1038/nn1533 · Cited by 129 · see citing works
Self-citationSlack and Slick KNaChannels Regulate the Accuracy of Timing of Auditory Neurons
Yang, B., Desai, R., & Kaczmarek, L. K. (2007) · Journal of Neuroscience · doi:10.1523/jneurosci.5308-06.2007 · Cited by 125 · see citing works
Self-citationThe medial nucleus of the trapezoid body: Comparative physiology
Kopp‐Scheinpflug, C., Tolnai, S., Malmierca, M. S., & Rübsamen, R. (2008) · Neuroscience · doi:10.1016/j.neuroscience.2008.01.088 · Cited by 123 · see citing works
Kanemasa, T., Gan, L., Perney, T. M., Wang, L., & Kaczmarek, L. K. (1995) · Journal of Neurophysiology · doi:10.1152/jn.1995.74.1.207 · Cited by 122 · see citing works
Self-citationEncoding of Timing in the Brain Stem Auditory Nuclei of Vertebrates
Oertel, D. (1997) · Neuron · doi:10.1016/s0896-6273(00)80388-8 · Cited by 112 · see citing works
Leão, R. N., Sun, H. Q., Leão, K. E., Berntson, A., Youssoufian, M., Paolini, A. G., Fyffe, R. E. W., & Walmsley, B. D. (2005) · The Journal of Physiology · doi:10.1113/jphysiol.2005.098780 · Cited by 111 · see citing works
Speech disturbances (cluttering) in mildly impaired males with the Martin‐Bell/fragile X syndrome
Hanson, D. M., Jackson, A. W., Hagerman, R. J., Opitz, J. M., & Reynolds, J. F. (1986) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320230114 · Cited by 103 · see citing works
von Hehn, C. A. A., Bhattacharjee, A., & Kaczmarek, L. K. (2004) · Journal of Neuroscience · doi:10.1523/jneurosci.4554-03.2004 · Cited by 98 · see citing works
Self-citationOzaita, A., Martone, M. E., Ellisman, M. H., & Rudy, B. (2002) · Journal of Neurophysiology · doi:10.1152/jn.2002.88.1.394 · Cited by 87 · see citing works
On BC1 RNA and the fragile X mental retardation protein
Iacoangeli, A., Rozhdestvensky, T. S., Dolzhanskaya, N., Tournier, B., Schütt, J., Brosius, J., Denman, R. B., Khandjian, E. W., Kindler, S., & Tiedge, H. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0710991105 · Cited by 83 · see citing works
Distribution of HCN1 and HCN2 in rat auditory brainstem nuclei
Koch, U., Braun, M., Kapfer, C., & Grothe, B. (2004) · European Journal of Neuroscience · doi:10.1111/j.0953-816x.2004.03456.x · Cited by 80 · see citing works
Kadner, A., & Berrebi, A. S. (2007) · Neuroscience · doi:10.1016/j.neuroscience.2007.11.008 · Cited by 74 · see citing works
Kaczmarek, L. K., Bhattacharjee, A., Desai, R., Gan, L., Song, P., von Hehn, C. A. A., Whim, M. D., & Yang, B. (2005) · Hearing Research · doi:10.1016/j.heares.2004.11.023 · Cited by 67 · see citing works
Self-citationBrew, H. M., & Forsythe, I. D. (2005) · Hearing Research · doi:10.1016/j.heares.2004.12.012 · Cited by 63 · see citing works
Elezgarai, I., Díez, J., Puente, N., Azkue, J. J., Benı́tez, R., Bilbao, A., Knöpfel, T., Doñate-Oliver, F., & Grandes, P. (2003) · Neuroscience · doi:10.1016/s0306-4522(03)00068-x · Cited by 61 · see citing works
Properties and expression of Kv3 channels in cerebellar Purkinje cells
Sacco, T., Luca, A. D., & Tempia, F. (2006) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2006.07.006 · Cited by 45 · see citing works
Strumbos, J. G., Polley, D. B., & Kaczmarek, L. K. (2010) · Neuroscience · doi:10.1016/j.neuroscience.2010.02.046 · Cited by 42 · see citing works
Self-citationThe neural basis of auditory temporal discrimination in girls with fragile X syndrome
Hall, S. S., Walter, E., Sherman, E., Hoeft, F., & Reiss, A. L. (2009) · Journal of Neurodevelopmental Disorders · doi:10.1007/s11689-009-9007-x · Cited by 35 · see citing works
Wang, L., Gan, L., Perney, T. M., Schwartz, I. R., & Kaczmarek, L. K. (1998) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.95.4.1882 · Cited by 31 · see citing works
Self-citationWhim, M. D., & Kaczmarek, L. K. (1998) · Journal of Neuroscience · doi:10.1523/jneurosci.18-22-09171.1998 · Cited by 27 · see citing works
Self-citationMiller (1999) · doi:10.1002/(sici)1096-8628(19990402)83:4
Flagged first, then by citations
Cited by
Sorted by citations
The translation of translational control by FMRP: therapeutic targets for FXS
Darnell, J. C., & Klann, E. (2013) · Nature Neuroscience · doi:10.1038/nn.3379 · Cited by 491 · see citing works
Linking neural activity and molecular oscillations in the SCN
Colwell, C. S. (2011) · Nature Reviews Neuroscience · doi:10.1038/nrn3086 · Cited by 474 · see citing works
Gribkoff, V. K., & Kaczmarek, L. K. (2016) · Neuropharmacology · doi:10.1016/j.neuropharm.2016.03.021 · Cited by 379 · see citing works
Self-citationDeng, 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
Dysregulation and restoration of translational homeostasis in fragile X syndrome
Richter, J. D., Bassell, G. J., & Klann, E. (2015) · Nature Reviews Neuroscience · doi:10.1038/nrn4001 · Cited by 275 · see citing works
Fragile X mental retardation protein and synaptic plasticity
Sidorov, M. S., Auerbach, B. D., & Bear, M. F. (2013) · Molecular Brain · doi:10.1186/1756-6606-6-15 · Cited by 243 · see citing works
The Calyx of Held Synapse: From Model Synapse to Auditory Relay
Borst, J. G. G., & van Hoeve, J. S. S. (2011) · Annual Review of Physiology · doi:10.1146/annurev-physiol-020911-153236 · Cited by 225 · see citing works
Kv3 Channels: Enablers of Rapid Firing, Neurotransmitter Release, and Neuronal Endurance
Kaczmarek, L. K., & Zhang, Y. (2017) · Physiological Reviews · doi:10.1152/physrev.00002.2017 · Cited by 194 · see citing works
Self-citationWang, J., Ethridge, L. E., Mosconi, M. W., White, S. P., Binder, D. K., Pedapati, E. V., Erickson, C. A., Byerly, M. J., & Sweeney, J. A. (2017) · Journal of Neurodevelopmental Disorders · doi:10.1186/s11689-017-9191-z · Cited by 176 · see citing works
Hays, S. A., Huber, K. M., & Gibson, J. R. (2011) · Journal of Neuroscience · doi:10.1523/jneurosci.3157-11.2011 · Cited by 167 · 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
Lee, H. Y., Ge, W., Huang, W. C., He, Y., Wang, G. X., Rowson-Baldwin, A., Smith, S. J., Jan, Y. N., & Jan, L. Y. (2011) · Neuron · doi:10.1016/j.neuron.2011.09.033 · Cited by 149 · see citing works
Deng, P., Sojka, D., & Klyachko, V. A. (2011) · Journal of Neuroscience · doi:10.1523/jneurosci.2021-11.2011 · Cited by 139 · see citing works
Altered auditory processing in a mouse model of fragile X syndrome
Rotschafer, S. E., & Razak, K. A. (2013) · Brain Research · doi:10.1016/j.brainres.2013.02.038 · Cited by 138 · see citing works
Myrick, L. K., Deng, P.-Y., Hashimoto, H., Oh, Y. M., Cho, Y., Poidevin, M. J., Suhl, J. A., Visootsak, J., Cavalli, V., Jin, P., et al. (2015) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1423094112 · Cited by 133 · see citing works
Sensory Processing Phenotypes in Fragile X Syndrome
Rais, M., Binder, D. K., Razak, K. A., & Ethell, I. M. (2018) · ASN NEURO · doi:10.1177/1759091418801092 · Cited by 128 · see citing works
Multifarious Functions of the Fragile X Mental Retardation Protein
Davis, J. K., & Broadie, K. S. (2017) · Trends in Genetics · doi:10.1016/j.tig.2017.07.008 · Cited by 118 · see citing works
Lovelace, J. W., Wen, T. H., Reinhard, S., Hsu, M. S., Sidhu, H., Ethell, I. M., Binder, D. K., & Razak, K. A. (2016) · Neurobiology of Disease · doi:10.1016/j.nbd.2016.02.002 · Cited by 115 · see citing works
ATP Synthase c-Subunit Leak Causes Aberrant Cellular Metabolism in Fragile X Syndrome
Licznerski, P., Park, H., Rolyan, H., Chen, R., Mnatsakanyan, N., Miranda, P., Graham, M. M., Wu, J., Cruz-Reyes, N., Mehta, N. A., et al. (2020) · Cell · doi:10.1016/j.cell.2020.07.008 · Cited by 114 · see citing works
Therapeutic Strategies in Fragile X Syndrome: From Bench to Bedside and Back
Gross, C., Hoffmann, A., Bassell, G. J., & Berry-Kravis, E. M. (2015) · Neurotherapeutics · doi:10.1007/s13311-015-0355-9 · Cited by 105 · see citing works
Therapeutic Strategies in Fragile X Syndrome: Dysregulated mGluR Signaling and Beyond
Groß, C., Berry‐Kravis, E., & Bassell, G. J. (2011) · Neuropsychopharmacology · doi:10.1038/npp.2011.137 · Cited by 103 · see citing works
Channelopathies in fragile X syndrome
Deng, P., & Klyachko, V. A. (2021) · Nature reviews. Neuroscience · doi:10.1038/s41583-021-00445-9 · Cited by 105 · see citing works
Targeted treatments for fragile X syndrome
Berry‐Kravis, E., Knox, A. T., & Hervey, C. M. (2011) · Journal of Neurodevelopmental Disorders · doi:10.1007/s11689-011-9074-7 · Cited by 103 · see citing works
Auditory Processing in Fragile X Syndrome
Rotschafer, S. E., & Razak, K. A. (2014) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2014.00019 · Cited by 103 · see citing works
Doll, C. A., & Broadie, K. S. (2014) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2014.00030 · Cited by 81 · see citing works
Kalmbach, B. E., Johnston, D., & Brager, D. H. (2015) · eNeuro · doi:10.1523/eneuro.0114-15.2015 · Cited by 82 · see citing works
Lovelace, J., Rais, M., Palacios, A. R., Shuai, X. S., Bishay, S., Popa, O., Pirbhoy, P. S., Binder, D., Nelson, D., Ethell, I. M., et al. (2019) · Cerebral Cortex · doi:10.1093/cercor/bhz141 · Cited by 72 · 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
Mnatsakanyan, N., & Jonas, E. A. (2020) · Journal of Molecular and Cellular Cardiology · doi:10.1016/j.yjmcc.2020.05.013 · Cited by 76 · see citing works
Routh, B. N., Johnston, D., & Brager, D. H. (2013) · Journal of Neuroscience · doi:10.1523/jneurosci.3256-13.2013 · Cited by 74 · see citing works
Impaired Critical Period Plasticity in Primary Auditory Cortex of Fragile X Model Mice
Kim, H., Gibboni, R., Kirkhart, C., & Bao, S. (2013) · The Journal of Neuroscience · doi:10.1523/jneurosci.3246-12.2013 · Cited by 74 · see citing works
Deng, P., & Klyachko, V. A. (2015) · The Journal of Physiology · doi:10.1113/jp271031 · Cited by 73 · see citing works
Deletion of Fmr1 alters function and synaptic inputs in the auditory brainstem
Rotschafer, S. E., Marshak, S., & Cramer, K. S. (2015) · PLOS ONE · doi:10.1371/journal.pone.0117266 · Cited by 72 · see citing works
Yang, Y., Arsenault, J., Bah, A., Krzeminski, M., Fekete, Á., Chao, O. Y.-H., Pacey, L. K. K., Wang, A. B., Forman-Kay, J. D., Hampson, D. R., et al. (2018) · Molecular Psychiatry · doi:10.1038/s41380-018-0240-0 · Cited by 70 · see citing works
Cellular distribution of the fragile X mental retardation protein in the mouse brain
Zorio, D. A. R., Jackson, C. M., Liu, Y., Rubel, E. W., & Wang, Y. (2016) · The Journal of Comparative Neurology · doi:10.1002/cne.24100 · Cited by 67 · see citing works
Ravanidis, S., Kattan, F.-G., & Doxakis, E. (2018) · International Journal of Molecular Sciences · doi:10.3390/ijms19082280 · Cited by 65 · see citing works
Mechanisms underlying auditory processing deficits in Fragile X syndrome
McCullagh, E., Rotschafer, S., Auerbach, B. D., Klug, A., Kaczmarek, L. K., Cramer, K. S., Kulesza, R., Razak, K. A., Lovelace, J., Lu, Y., et al. (2020) · The FASEB Journal · doi:10.1096/fj.201902435r · Cited by 64 · see citing works
Self-citationChannelopathies and dendritic dysfunction in fragile X syndrome
Brager, D. H., & Johnston, D. (2014) · Brain Research Bulletin · doi:10.1016/j.brainresbull.2014.01.002 · Cited by 64 · see citing works
van Welie, I., & du Lac, S. (2011) · Journal of Neurophysiology · doi:10.1152/jn.00058.2011 · Cited by 64 · see citing works
Deng, P., Carlin, D., Oh, Y. M., Myrick, L. K., Warren, S. T., Cavalli, V., & Klyachko, V. A. (2018) · Journal of Neuroscience · doi:10.1523/jneurosci.1593-18.2018 · Cited by 60 · see citing works
Modulators of Kv3 Potassium Channels Rescue the Auditory Function of Fragile X Mice
El‐Hassar, L., Song, L., Tan, W. J. T., Large, C., Alvaro, G. S., Santos-Sacchi, J., & Kaczmarek, L. (2019) · Journal of Neuroscience · doi:10.1523/jneurosci.0839-18.2019 · Cited by 60 · see citing works
Self-citationGarcia-Pino, E., Gessele, N., & Koch, U. (2017) · The Journal of Neuroscience · doi:10.1523/jneurosci.2310-16.2017 · Cited by 58 · see citing works
Disrupted-in-schizophrenia 1 regulates transport of ITPR1 mRNA for synaptic plasticity
Tsuboi, D., Kuroda, K., Tanaka, M., Namba, T., Iizuka, Y., Taya, S., Shinoda, T., Hikita, T., Muraoka, S., Iizuka, M., et al. (2015) · Nature Neuroscience · doi:10.1038/nn.3984 · Cited by 57 · see citing works
Degraded speech sound processing in a rat model of fragile X syndrome
Engineer, C., Centanni, T., Im, K. W., Rahebi, K. C., Buell, E. P., & Kilgard, M. (2014) · Brain Research · doi:10.1016/j.brainres.2014.03.049 · Cited by 44 · see citing works
Domanski, 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
Malik, R., Pai, E. L.-L., Rubin, A. N., Stafford, A. M., Angara, K., Minasi, P., Rubenstein, J. L. R., Sohal, V. S., & Vogt, D. L. (2019) · Nature Communications · doi:10.1038/s41467-019-12962-4 · Cited by 55 · see citing works
Hyperexcitability and homeostasis in fragile X Syndrome.
Liu, X., Kumar, V., Tsai, N.-P., & Auerbach, B. D. (2022) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2021.805929 · Cited by 53 · see citing works
Homeostatic 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
Fragile X syndrome: From targets to treatments
Wijetunge, L. S., Chattarji, S., Wyllie, D. J. A., & Kind, P. C. (2012) · Neuropharmacology · doi:10.1016/j.neuropharm.2012.11.028 · Cited by 49 · see citing works
Keeping Excitation–Inhibition Ratio in Balance
Kirischuk, S. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23105746 · Cited by 48 · see citing works
Intellectual Disability and Potassium Channelopathies: A Systematic Review
Kessi, M., Chen, B., Peng, J., Tang, Y., Olatoutou, E., He, F. P., Yang, L., & Yin, F. (2020) · Frontiers in Genetics · doi:10.3389/fgene.2020.00614 · Cited by 48 · see citing works
From FMRP Function to Potential Therapies for Fragile X Syndrome
Sethna, F., Moon, C., & Wang, H. (2013) · Neurochemical Research · doi:10.1007/s11064-013-1229-3 · Cited by 47 · see citing works
Do Astrocytes Play a Role in Intellectual Disabilities?
Cresto, N., Pillet, L., Billuart, P., & Rouach, N. (2019) · Trends in Neurosciences · doi:10.1016/j.tins.2019.05.011 · Cited by 49 · see citing works
Franco, L. M., Okray, Z., Linneweber, G. A., Hassan, B. A., & Yaksi, E. (2017) · Current Biology · doi:10.1016/j.cub.2017.02.065 · Cited by 45 · see citing works
Physiological modulators of Kv3.1 channels adjust firing patterns of auditory brain stem neurons
Brown, M. R., El‐Hassar, L., Zhang, Y., Alvaro, G. S., Large, C. H., & Kaczmarek, L. K. (2016) · Journal of Neurophysiology · doi:10.1152/jn.00174.2016 · Cited by 44 · see citing works
Self-citationPotassium channel modulation and auditory processing
Brown, M. R., & Kaczmarek, L. K. (2011) · Hearing Research · doi:10.1016/j.heares.2011.03.004 · Cited by 42 · see citing works
Self-citationmGluR5 Negative Modulators for Fragile X: Treatment Resistance and Persistence
Stoppel, D. C., McCamphill, P. K., Senter, R. K., Heynen, A. J., & Bear, M. F. (2021) · Frontiers in Psychiatry · doi:10.3389/fpsyt.2021.718953 · Cited by 41 · see citing works
Abnormal neuronal morphology and neurochemistry in the auditory brainstem of Fmr1 knockout rats.
Ruby, K., Falvey, K., & Kulesza, R. J. (2015) · Neuroscience · doi:10.1016/j.neuroscience.2015.06.061 · Cited by 41 · see citing works
Wang, Y., Sakano, H., Beebe, K., Brown, M. R., de Laat, R., Bothwell, M., Kulesza, R. J., & Rubel, E. W. (2014) · Journal of Comparative Neurology · doi:10.1002/cne.23520 · Cited by 40 · see citing works
Self-citationBataveljic, D., Pivonkova, H., de Concini, V., Hébert, B., Ezan, P., Briault, S., Bemelmans, A.-P., Pichon, J., Menuet, A., & Rouach, N. (2024) · Nature Communications · doi:10.1038/s41467-024-47681-y · Cited by 40 · see citing works
Chmielewska, J. J., Kuźniewska, B., Miłek, J., Urbanska, K., & Dziembowska, M. (2018) · Molecular Neurobiology · doi:10.1007/s12035-018-1243-1 · Cited by 44 · see citing works
Doll, C. A., & Broadie, K. S. (2016) · Neurobiology of Disease · doi:10.1016/j.nbd.2016.02.006 · Cited by 39 · see citing works
Imbalance of synaptic actin dynamics as a key to fragile X syndrome?
Michaelsen‐Preusse, K., Feuge, J., & Körte, M. (2018) · The Journal of Physiology · doi:10.1113/jp275571 · Cited by 35 · see citing works
Lü, P., Chen, X., Feng, Y., Zeng, Q., Jiang, C., Zhu, X., Fan, G., & Xue, Z. (2016) · Science China Life Sciences · doi:10.1007/s11427-016-0194-6 · Cited by 32 · see citing works
Nguyen, A. O., Binder, D. K., Ethell, I. M., & Razak, K. A. (2020) · Journal of Neurophysiology · doi:10.1152/jn.00706.2019 · Cited by 28 · see citing works
Distribution of fragile X mental retardation protein in the human auditory brainstem
Beebe, K., Wang, Y., & Kulesza, R. J. (2014) · Neuroscience · doi:10.1016/j.neuroscience.2014.05.006 · Cited by 30 · see citing works
Auditory Dysfunction in Animal Models of Autism Spectrum Disorder
Castro, A. C., & Monteiro, P. (2022) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2022.845155 · Cited by 34 · see citing works
Choudhury, N., Linley, D. M., Richardson, A., Anderson, M., Robinson, S. W., Marra, V., Ciampani, V., Walter, S. M., Kopp‐Scheinpflug, C., Steinert, J. R., et al. (2020) · The Journal of Physiology · doi:10.1113/jp279668 · Cited by 29 · see citing works
Ohashi, R., & Shiina, N. (2020) · Biomolecules · doi:10.3390/biom10020167 · Cited by 28 · see citing works
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., et al. (2020) · Molecular Autism · doi:10.1186/s13229-020-00351-4 · Cited by 28 · see citing works
Wang, T., de Kok, L. G. C., Willemsen, R., Elgersma, Y., & Borst, J. G. G. (2015) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2015.00234 · Cited by 28 · see citing works
FMRP-Driven Neuropathology in Autistic Spectrum Disorder and Alzheimer's disease: A Losing Game
Bleuzé, L., Triaca, V., & Borreca, A. (2021) · Frontiers in Molecular Biosciences · doi:10.3389/fmolb.2021.699613 · Cited by 27 · see citing works
McCullagh, E. A., Salcedo, E. E., Huntsman, M. M., & Klug, A. (2017) · The Journal of Comparative Neurology · doi:10.1002/cne.24290 · Cited by 26 · see citing works
Characterization of Auditory and Binaural Spatial Hearing in a Fragile X Syndrome Mouse Model
McCullagh, E. A., Poleg, S., Greene, N. T., Huntsman, M. M., Tollin, D. J., & Klug, A. (2020) · eNeuro · doi:10.1523/eneuro.0300-19.2019 · Cited by 26 · see citing works
Lee, H. Y., & Jan, L. Y. (2012) · Current Opinion in Neurobiology · doi:10.1016/j.conb.2012.03.010 · Cited by 25 · see citing works
Targeted pharmacological treatment of autism spectrum disorders: fragile X and Rett syndromes
Wang, H., Pati, S. B. B., Pozzo‐Miller, L. D., & Doering, L. C. (2015) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2015.00055 · Cited by 25 · see citing works
Tuning of synaptic responses: an organizing principle for optimization of neural circuits
O’Donnell, C., & Nolan, M. F. (2010) · Trends in Neurosciences · doi:10.1016/j.tins.2010.10.003 · Cited by 24 · see citing works
Advances in understanding fragile X syndrome and related disorders
Rooms, L., & Kooy, R. F. (2011) · Current Opinion in Pediatrics · doi:10.1097/mop.0b013e32834c7f1a · Cited by 22 · see citing works
Faulkner, I. E., Pajak, R. Z., Harte, M., Glazier, J. D., & Hager, R. (2024) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2024.1449151 · Cited by 22 · see citing works
Disruption of GpI mGluR-Dependent Cav2.3 Translation in a Mouse Model of Fragile X Syndrome
Gray, E. E., Murphy, J. G., Liu, Y., Trang, I., Tabor, G. T., Lin, L., & Hoffman, D. A. (2019) · Journal of Neuroscience · doi:10.1523/jneurosci.1443-17.2019 · Cited by 21 · see citing works
Failed Stabilization for Long-Term Potentiation in the Auditory Cortex of Fmr1 Knockout Mice
Yang, S., Yang, S., Park, J.-S., Kirkwood, A., & Bao, S.-W. (2014) · PLoS ONE · doi:10.1371/journal.pone.0104691 · Cited by 23 · see citing works
Increasing our understanding of human cognition through the study of fragile X syndrome
Cook, D., Nuro, E., & Murai, K. K. (2013) · Developmental Neurobiology · doi:10.1002/dneu.22096 · Cited by 20 · see citing works
Mechanisms Driving the Emergence of Neuronal Hyperexcitability in Fragile X Syndrome
Bülow, P., Segal, M., & Bassell, G. J. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23116315 · Cited by 19 · see citing works
Polysomnographic Findings in Fragile X Syndrome Children with EEG Abnormalities
Carotenuto, M., Roccella, M., Pisani, F., Matricardi, S., Verrottı, A., Farello, G., Operto, F. F., Bitetti, I., Precenzano, F., Messina, G., et al. (2019) · Behavioural Neurology · doi:10.1155/2019/5202808 · Cited by 19 · see citing works
Pandina, G., Ring, R. H., Bangerter, A., & Ness, S. L. (2020) · Psychiatric Clinics of North America · doi:10.1016/j.psc.2020.08.003 · Cited by 18 · see citing works
Berry‐Kravis, E. (2022) · Journal of Child Neurology · doi:10.1177/08830738221089740 · Cited by 18 · see citing works
Mechanisms regulating input-output function and plasticity of neurons in the absence of FMRP
Booker, S. A., & Kind, P. C. (2021) · Brain Research Bulletin · doi:10.1016/j.brainresbull.2021.06.025 · Cited by 16 · see citing works
Holley, A. J., Shedd, A., Boggs, A. E., Lovelace, J. W., Erickson, C. A., Groß, C., Jankovic, M. J., Razak, K. A., Huber, K. M., & Gibson, J. R. (2022) · Neurobiology of Disease · doi:10.1016/j.nbd.2022.105767 · Cited by 16 · see citing works
Pandina, G., Ring, R. H., Bangerter, A., & Ness, S. L. (2020) · Child and Adolescent Psychiatric Clinics of North America · doi:10.1016/j.chc.2019.12.004 · Cited by 16 · see citing works
Myelination Deficits in the Auditory Brainstem of a Mouse Model of Fragile X Syndrome
Lucas, A., Poleg, S., Klug, A., & McCullagh, E. (2021) · bioRxiv · doi:10.3389/fnins.2021.772943 · Cited by 12 · see citing works
Wang, X.-Y., Kohl, A., Yu, X.-Y., Zorio, D., Klar, A., Sela-Donenfeld, D., & Wang, Y. (2020) · Development · doi:10.1242/dev.188797 · Cited by 13 · see citing works
Gradients and Modulation of K+ Channels Optimize Temporal Accuracy in Networks of Auditory Neurons
Kaczmarek, L. K. (2012) · PLoS Computational Biology · doi:10.1371/journal.pcbi.1002424 · Cited by 13 · see citing works
Self-citationAxonal and presynaptic FMRP: localization, signal, and functional implications
Wang, X.-Y., Sela-Donenfeld, D., & Wang, Y. (2023) · Hearing Research · doi:10.1016/j.heares.2023.108720 · Cited by 11 · see citing works
Lu, Y. (2019) · Brain Research · doi:10.1016/j.brainres.2019.04.006 · Cited by 14 · see citing works
Kalmbach, B. E., & Brager, D. H. (2020) · Journal of Neurophysiology · doi:10.1152/jn.00494.2020 · Cited by 11 · see citing works
The role of altered translation in intellectual disability and epilepsy
Malone, T. J., & Kaczmarek, L. K. (2022) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2022.102267 · Cited by 11 · see citing works
Self-citationModulation of potassium conductances optimizes fidelity of auditory information
Kaczmarek, L. K. (2023) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.2216440120 · Cited by 11 · see citing works
Self-citationvan Andel, D. M., Sprengers, J. J., Keijzer‐Veen, M. G., Schulp, A. J. A., Lillien, M. R., Scheepers, F. E., & Bruining, H. (2022) · Frontiers in Psychiatry · doi:10.3389/fpsyt.2022.780281 · Cited by 11 · see citing works
Luque, M. A., Morcuende, S., Torres, B., & Herrero, L. (2024) · The Journal of Physiology · doi:10.1113/jp285244 · Cited by 9 · see citing works
Dwivedi, D., Chattarji, S., & Bhalla, U. S. (2019) · eNeuro · doi:10.1523/eneuro.0217-19.2019 · Cited by 9 · see citing works
- Authors
- Strumbos, J. G., Brown, M. R., Kronengold, J., Polley, D. B., & Kaczmarek, L. K.
- Affiliations
- Yale University (US)
- Massachusetts Eye and Ear Infirmary (US)
- Corresponding author
- Kaczmarek, Leonard K.
- Venue
- The Journal of Neuroscience
- Publisher
- Society for Neuroscience
- Published
- 2010-08-04
- Volume
- 30
- Issue
- 31
- Pages
- 10263-10271
- ISSN
- 0270-6474 (ISSN-L)
- 1529-2401
- PMC
- PMC3485078
- OpenAlex
- W2034460847
- Cited by
- 135
- References
- 56
- Citations by year
- 2026: 1 · 2025: 4 · 2024: 5 · 2023: 4 · 2022: 11 · 2021: 7 · 2020: 14 · 2019: 14 · 2018: 10 · 2017: 10 · 2016: 9 · 2015: 12 · 2014: 7 · 2013: 9 · 2012: 5
- FWCI
- 4.85
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Neuroscience (publishedVersion, cc-by-nc-sa)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- FRAXA Research Foundation
- National Institutes of Health
- National Institute on Deafness and Other Communication Disorders (DC001919, R01DC009836, R03DC009488, DC009488, R01DC001919)
- 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 › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Ion channel regulation and function
- Keywords
- FMRP, voltage-gated potassium channels, medial nucleus of the trapezoid body, Fmr1 knockout mice, synaptic plasticity, translation repression, RNA-binding proteins
- MeSH terms
- Acoustic Stimulation, Animals, Auditory Pathways, Brain Stem, Immunohistochemistry, Male, Neurons, RNA, Messenger, Sound Localization, Blotting, Western, Evoked Potentials, Auditory, Brain Stem, Mice, Knockout, Mice, Shaw Potassium Channels, Fragile X Messenger Ribonucleoprotein 1
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 ›
Are you an author, or do you see a problem with this page? Request a review
