Deletion of Fmr1 alters function and synaptic inputs in the auditory brainstem
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Fragile X Syndrome (FXS), a neurodevelopmental disorder, is the most prevalent single-gene cause of autism spectrum disorder. Autism has been associated with impaired auditory processing, abnormalities in the auditory brainstem response (ABR), and reduced cell number and size in the auditory brainstem nuclei. FXS is characterized by elevated cortical responses to sound stimuli, with some evidence for aberrant ABRs. Here, we assessed ABRs and auditory brainstem anatomy in Fmr1-/- mice, an animal model of FXS. We found that Fmr1-/- mice showed elevated response thresholds to both click and tone stimuli. Amplitudes of ABR responses were reduced in Fmr1-/- mice for early peaks of the ABR. The growth of the peak I response with sound intensity was less steep in mutants that in wild type mice. In contrast, amplitudes and response growth in peaks IV and V did not differ between these groups. We did not observe differences in peak latencies or in interpeak latencies. Cell size was reduced in Fmr1-/- mice in the ventral cochlear nucleus (VCN) and in the medial nucleus of the trapezoid body (MNTB). We quantified levels of inhibitory and excitatory synaptic inputs in these nuclei using markers for presynaptic proteins. We measured VGAT and VGLUT immunolabeling in VCN, MNTB, and the lateral superior olive (LSO). VGAT expression in MNTB was significantly greater in the Fmr1-/- mouse than in wild type mice. Together, these observations demonstrate that FXS affects peripheral and central aspects of hearing and alters the balance of excitation and inhibition in the auditory brainstem.
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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 Rotschafer et al., 2015
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Additional file 1 of Auditory processing in rodent models of autism: a systematic review
Wilde, M., Constantin, L., Thorne, P. R., Montgomery, J. M., Scott, E. K., & Cheyne, J. E. (2022) · Figshare · doi:10.6084/m9.figshare.20745164
BKCa channels as therapeutic targets in neurodevelopmental disorders : focus on acoustic dysfunction
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- Authors
- Rotschafer, S. E., Marshak, S., & Cramer, K. S.
- Affiliations
- University of California, Irvine (US)
- Corresponding author
- Cramer, Karina S.
- ORCID
- Cramer, Karina S.
- Venue
- PLOS ONE
- Publisher
- Public Library of Science (PLoS)
- Published
- 2015-02-13
- Volume
- 10
- Issue
- 2
- Pages
- e0117266
- ISSN
- 1932-6203 (ISSN-L)
- PMC
- PMC4332492
- OpenAlex
- W2089345123
- Cited by
- 72
- References
- 61
- Citations by year
- 2026: 1 · 2025: 5 · 2024: 5 · 2023: 8 · 2022: 15 · 2021: 4 · 2020: 6 · 2019: 10 · 2018: 6 · 2017: 6 · 2016: 5 · 2015: 1
- FWCI
- 4.10
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- PLoS ONE (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Funders
- National Institutes of Health
- University of California, Irvine
- National Institute on Deafness and Other Communication Disorders (P30DC008369, T32DC010775)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Neuroscience › Sensory Systems › Hearing, Cochlea, Tinnitus, Genetics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Neuroscience and Neuropharmacology Research
- Keywords
- fragile X syndrome, Fmr1 knockout mice, auditory brainstem, auditory brainstem response, ventral cochlear nucleus, medial nucleus of the trapezoid body, lateral superior olive, synaptic inhibition, excitatory synaptic input, vesicular GABA transporter, vesicular glutamate transporter, excitation-inhibition balance, auditory processing, autism spectrum disorder
- MeSH terms
- Acoustic Stimulation, Animals, Auditory Pathways, Auditory Threshold, Brain Stem, Fragile X Syndrome, gamma-Aminobutyric Acid, Evoked Potentials, Auditory, Brain Stem, Gene Deletion, Cochlear Nucleus, Mice, Knockout, Mice, Fragile X Messenger Ribonucleoprotein 1, Synaptic Potentials, GABAergic Neurons
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