ArticleBrain research2014
Degraded speech sound processing in a rat model of fragile X syndrome.
Article in Brain research, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 1 of them a synthesis that pooled it.
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Who cites it
29 citing papers in PubMed, 1 synthesis or guideline pooled it, 59 citations in OpenAlex.
- Auditory processing in rodent models of autism: a systematic review.Journal of neurodevelopmental disorders · 2022Pooled it
- Foxg1 gene mutation impairs auditory cortex response and reduces sound tolerance.Cerebral cortex (New York, N.Y. : 1991) · 2025Article
- Use of a predictor cue during a speech sound discrimination task in a Cntnap2 knockout rat model of autism.PloS one · 2025Article
- Pairing tones with vagus nerve stimulation improves brain stem responses to speech in the valproic acid model of autism.Journal of neurophysiology · 2024Article
- Topography and Ensemble Activity in the Auditory Cortex of a Mouse Model of Fragile X Syndrome.eNeuro · 2024Article
- Axonal and presynaptic FMRP: Localization, signal, and functional implications.Hearing research · 2023Review
- Mouse models of fragile X-related disorders.Disease models & mechanisms · 2023Review
- Hyperexcitable and immature-like neuronal activity in the auditory cortex of adult rats lacking the language-linked CNTNAP2 gene.Cerebral cortex (New York, N.Y. : 1991) · 2022Article
- Auditory Dysfunction in Animal Models of Autism Spectrum Disorder.Frontiers in molecular neuroscience · 2022Review
- Absence of the Fragile X messenger ribonucleoprotein alters response patterns to sounds in the auditory midbrain.Frontiers in neuroscience · 2022Article
- Gamma power abnormalities in a Fmr1-targeted transgenic rat model of fragile X syndrome.Scientific reports · 2020Article
- Reduced axonal caliber and structural changes in a rat model of Fragile X syndrome with a deletion of a K-Homology domain of Fmr1.Translational psychiatry · 2020Article
- Deletion of the KH1 Domain of Fmr1 Leads to Transcriptional Alterations and Attentional Deficits in Rats.Cerebral cortex (New York, N.Y. : 1991) · 2019Article
- The Application of Adeno-Associated Viral Vector Gene Therapy to the Treatment of Fragile X Syndrome.Brain sciences · 2019Review
- Modeling Fragile X Syndrome inFrontiers in molecular neuroscience · 2018Review
- Shank3-deficient rats exhibit degraded cortical responses to sound.Autism research : official journal of the International Society for Autism Research · 2018Article
- Of Men and Mice: Modeling the Fragile X Syndrome.Frontiers in molecular neuroscience · 2018Article
- Loss of FMRP Impaired Hippocampal Long-Term Plasticity and Spatial Learning in Rats.Frontiers in molecular neuroscience · 2017Article
- Sensory hypo-excitability in a rat model of fetal development in Fragile X Syndrome.Scientific reports · 2016Article
- Knockdown of Dyslexia-Gene Dcdc2 Interferes with Speech Sound Discrimination in Continuous Streams.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2016Article
Corrections and comments
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
Abstract
Fragile X syndrome is the most common inherited form of intellectual disability and the leading genetic cause of autism. Impaired phonological processing in fragile X syndrome interferes with the development of language skills. Although auditory cortex responses are known to be abnormal in fragile X syndrome, it is not clear how these differences impact speech sound processing. This study provides the first evidence that the cortical representation of speech sounds is impaired in Fmr1 knockout rats, despite normal speech discrimination behavior. Evoked potentials and spiking activity in response to speech sounds, noise burst trains, and tones were significantly degraded in primary auditory cortex, anterior auditory field and the ventral auditory field. Neurometric analysis of speech evoked activity using a pattern classifier confirmed that activity in these fields contains significantly less information about speech sound identity in Fmr1 knockout rats compared to control rats. Responses were normal in the posterior auditory field, which is associated with sound localization. The greatest impairment was observed in the ventral auditory field, which is related to emotional regulation. Dysfunction in the ventral auditory field may contribute to poor emotional regulation in fragile X syndrome and may help explain the observation that later auditory evoked responses are more disturbed in fragile X syndrome compared to earlier responses. Rodent models of fragile X syndrome are likely to prove useful for understanding the biological basis of fragile X syndrome and for testing candidate therapies.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.