The Fragile X mental retardation protein regulates matrix metalloproteinase 9 mRNA at synapses.
Open accessAbstract
Activity-dependent protein synthesis at synapses is dysregulated in the Fragile X syndrome (FXS). This process contributes to dendritic spine dysmorphogenesis and synaptic dysfunction in FXS. Matrix Metalloproteinase 9 (MMP-9) is an enzyme involved in activity-dependent reorganization of dendritic spine architecture and was shown to regulate spine morphology in a mouse model of FXS, the Fmr1 knock-out mice. Here we show that MMP-9 mRNA is part of the FMRP complex and colocalizes in dendrites. In the absence of FMRP MMP-9 mRNA translation is increased at synapses, suggesting that this mechanism contributes to the increased metalloproteinase level at synapses of Fmr1 knock-out mice. We propose that such a local effect can contribute to the aberrant dendritic spine morphology observed in the Fmr1 knock-out mice and in patients with FXS.
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MMP-9 upregulation in FXS can likely be attributed to loss of the Fmr1 gene.
“Unlike FXS, where MMP-9 upregulation can likely be attributed to loss of the Fmr1 gene (Janusz et al., 2013)”
From The Perineuronal ‘Safety’ Net? Perineuronal Net Abnormalities in Neurological Disorders (2018) · cites this paper as Janusz et al., 2013
MMP-9 translation is negatively regulated by FMRP.
“MMP-9 is an endopeptidase that can cleave extracellular matrix (Ethell and Ethell, 2007) and its translation is negatively regulated by FMRP (Janusz et al., 2013).”
From Matrix metalloproteinase-9 deletion rescues auditory evoked potential habituation deficit in a mouse model of Fragile X Syndrome (2016) · cites this paper as Janusz et al., 2013
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- Authors
- Janusz, A., Miłek, J., Perycz, M., Pacini, L., Bagni, C., Kaczmarek, L., & Dziembowska, M.
- Affiliations
- University of Rome Tor Vergata (IT)
- Instytut Biologii Doświadczalnej im. Marcelego Nenckiego (PL)
- International Institute of Molecular and Cell Biology (PL)
- ORCID
- Venue
- The Journal of Neuroscience
- Publisher
- Society for Neuroscience
- Published
- 2013-11-13
- Volume
- 33
- Issue
- 46
- Pages
- 18234-18241
- ISSN
- 0270-6474 (ISSN-L)
- 1529-2401
- PMC
- PMC6619756
- OpenAlex
- W2147050348
- Cited by
- 112
- References
- 38
- Citations by year
- 2025: 3 · 2024: 6 · 2023: 6 · 2022: 9 · 2021: 9 · 2020: 5 · 2019: 8 · 2018: 15 · 2017: 15 · 2016: 9 · 2015: 15 · 2014: 12
- FWCI
- 7.94
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Neuroscience (publishedVersion, cc-by-nc-sa)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Cineca Institutional Research Information System (Tor Vergata University) (submittedVersion)
- Funders
- Compagnia di San Paolo
- Fundacja na rzecz Nauki Polskiej
- 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 › RNA Research and Splicing
- Keywords
- fragile X syndrome, FMRP, MMP-9, dendritic spine morphology, synaptic dysfunction, mRNA translation, synapses, dendrites
- MeSH terms
- Animals, Dendrites, Female, Hippocampus, RNA, Messenger, Synapses, Mice, Knockout, Matrix Metalloproteinase 9, Mice, Rats, Fragile X Messenger Ribonucleoprotein 1
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