Genetic removal of matrix metalloproteinase 9 rescues the symptoms of fragile X syndrome in a mouse model
Open accessAbstract
Fmr1 knock-out (ko) mice display key features of fragile X syndrome (FXS), including delayed dendritic spine maturation and FXS-associated behaviors, such as poor socialization, obsessive-compulsive behavior, and hyperactivity. Here we provide conclusive evidence that matrix metalloproteinase-9 (MMP-9) is necessary to the development of FXS-associated defects in Fmr1 ko mice. Genetic disruption of Mmp-9 rescued key aspects of Fmr1 deficiency, including dendritic spine abnormalities, abnormal mGluR5-dependent LTD, as well as aberrant behaviors in open field and social novelty tests. Remarkably, MMP-9 deficiency also corrected non-neural features of Fmr1 deficiency-specifically macroorchidism-indicating that MMP-9 dysregulation contributes to FXS-associated abnormalities outside the CNS. Further, MMP-9 deficiency suppressed elevations of Akt, mammalian target of rapamycin, and eukaryotic translation initiation factor 4E phosphorylation seen in Fmr1 ko mice, which are also associated with other autistic spectrum disorders. These findings establish that MMP-9 is critical to the mechanisms responsible for neural and non-neural aspects of the FXS phenotype.
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MMP-9 is elevated in the cortex and hippocampus of both humans with Fragile X Syndrome and Fmr1 knockout mice.
“MMP-9 is elevated in the cortex and hippocampus of both humans with FXS and Fmr1 KO mice (Sidhu et al., 2014; Gkogkas et al., 2014).”
From Matrix metalloproteinase-9 deletion rescues auditory evoked potential habituation deficit in a mouse model of Fragile X Syndrome (2016) · cites this paper as Sidhu et al., 2014
Genetic deletion of Mmp-9 in Fmr1 knockout mice reverses some molecular and physiological hippocampal deficits and Fragile X Syndrome-associated behaviors.
“Genetic deletion of Mmp-9 in Fmr1 KO mice reverses some molecular and physiological hippocampal deficits and FXS-associated behaviors (Sidhu et al., 2014).”
From Matrix metalloproteinase-9 deletion rescues auditory evoked potential habituation deficit in a mouse model of Fragile X Syndrome (2016) · cites this paper as Sidhu et al., 2014
Deleting MMP-9 from Fmr1 KO mice reverses molecular and synaptic hippocampal deficits and some FXS-associated behaviors (Sidhu et al., 2014).
“Deletion of MMP-9 from Fmr1 KO mice results in reversal of molecular and synaptic deficits in the hippocampus of Fmr1 KO mice as well as some FXS-associated behaviors (Sidhu et al., 2014).”
From Matrix metalloproteinase-9 deletion rescues auditory evoked potential habituation deficit in a mouse model of Fragile X Syndrome (2016) · cites this paper as Sidhu et al., 2014
MMP-9 levels are high in cortex and hippocampus of humans with FXS and Fmr1 KO mice (Sidhu et al., 2014; Gkogkas et al., 2014).
“MMP-9 levels are high in the cortex and hippocampus of humans with FXS and Fmr1 KO mice (Sidhu et al., 2014; Gkogkas et al., 2014).”
From Matrix metalloproteinase-9 deletion rescues auditory evoked potential habituation deficit in a mouse model of Fragile X Syndrome (2016) · cites this paper as Sidhu et al., 2014
Removing MMP-9 reverses multiple FXS phenotypes in Fmr1 knock-out mice, among them poor habituation of auditory responses.
“MMP-9 deletion reverses several FXS phenotypes in Fmr1 KO mice (Sidhu et al. 2014), including impaired auditory response habituation (Lovelace et al. 2016).”
From Genetic Reduction of Matrix Metalloproteinase-9 Promotes Formation of Perineuronal Nets Around Parvalbumin-Expressing Interneurons and Normalizes Auditory Cortex Responses in Developing Fmr1 Knock-Out Mice (2018) · cites this paper as Sidhu et al. 2014
Similar sensory deficits are observed in adult Fmr1 knock-out mice and are reversed by genetic deletion of MMP-9.
“Similar sensory deficits are also observed in adult Fmr1 knock-out (KO) mice and are reversed by genetic deletion of Matrix Metalloproteinase-9 (MMP-9) through yet unknown mechanisms.”
From Genetic Reduction of Matrix Metalloproteinase-9 Promotes Formation of Perineuronal Nets Around Parvalbumin-Expressing Interneurons and Normalizes Auditory Cortex Responses in Developing Fmr1 Knock-Out Mice (2018) · cites this paper as Sidhu et al. 2014
Genetic deletion of MMP-9 rescues several other fragile X syndrome phenotypes, including improper dendritic spine maturation, enhanced mGluR5-dependent long-term depression, abnormal social behavior, and macroorchidism.
“Genetic deletion of MMP-9 rescues several other FXS phenotypes, including improper dendritic spine maturation, enhanced mGluR5-dependent-LTD, abnormal social behavior and macroorchidism (Sidhu et al. 2014)”
From Genetic Reduction of Matrix Metalloproteinase-9 Promotes Formation of Perineuronal Nets Around Parvalbumin-Expressing Interneurons and Normalizes Auditory Cortex Responses in Developing Fmr1 Knock-Out Mice (2018) · cites this paper as Sidhu et al. 2014
MMP-9 levels are elevated in fragile X syndrome human brains and in the hippocampus of Fmr1 knockout mice.
“MMP-9 levels are elevated in FXS human brains and the hippocampus of Fmr1 KO mice (Dziembowska et al., 2013;Sidhu et al. 2014;Gkogkas et al. 2014).”
From Genetic Reduction of Matrix Metalloproteinase-9 Promotes Formation of Perineuronal Nets Around Parvalbumin-Expressing Interneurons and Normalizes Auditory Cortex Responses in Developing Fmr1 Knock-Out Mice (2018) · cites this paper as Sidhu et al. 2014
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- Authors
- Sidhu, H., Dansie, L. E., Hickmott, P. W., Ethell, D. W., & Ethell, I. M.
- Affiliations
- University of California, Riverside (US)
- Western University of Health Sciences (US)
- Venue
- Journal of Neuroscience
- Publisher
- Society for Neuroscience
- Published
- 2014-07-23
- Volume
- 34
- Issue
- 30
- Pages
- 9867-9879
- ISSN
- 0270-6474 (ISSN-L)
- 1529-2401
- PMC
- PMC4107404
- OpenAlex
- W2083945553
- Cited by
- 155
- References
- 90
- Citations by year
- 2026: 2 · 2025: 5 · 2024: 6 · 2023: 13 · 2022: 13 · 2021: 15 · 2020: 6 · 2019: 14 · 2018: 15 · 2017: 22 · 2016: 18 · 2015: 23 · 2014: 3
- FWCI
- 14.41
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Journal of Neuroscience (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- Funders
- FRAXA Research Foundation
- California Institute for Regenerative Medicine
- National Institutes of Health
- National Institute of Mental Health (R01MH067121, MH 67121)
- 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 › Autism Spectrum Disorder Research
- Keywords
- fragile X syndrome, MMP-9, Fmr1 knockout mice, dendritic spine maturation, social behavior, macroorchidism, Akt signaling, mTOR signaling, eIF4E phosphorylation, autism spectrum disorder, genetic rescue
- MeSH terms
- Animals, Animals, Newborn, Cells, Cultured, Disease Models, Animal, Fragile X Syndrome, Male, Phenotype, Mice, Knockout, Matrix Metalloproteinase 9, Mice, Mice, 129 Strain
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