Fragile X Syndrome – Fmr1 KO Mouse Model

Accelerate the availability of targeted FXS therapies with the well-characterized and translationally relevant in vivo Fmr1 KO model

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Fmr1 KO Key Model Characteristics

Fragile X Syndrome (FXS) is the most prevalent inherited cause of mild-to-severe cause of intellectual disability and the most common monogenic cause of autism spectrum disorder (ASD). FXS is caused by mutations in the fragile X mental retardation protein 1 (FMR1) gene, resulting in the deficiency of the FMRP protein that plays a key role in synaptogenesis, as well as synaptic plasticity and architecture. The Fmr1 KO mouse model replicates many phenotypic features of human FXS, and therefore, represents a highly translationally relevant mouse model to study efficacy of novel disease-modifying treatments for FXS. 

InnoSer is committed to helping industry innovators to accelerate the availability of FXS modifying-treatment to patients through our extensive experience in carrying out efficacy studies in the Fmr1 KO mouse model. Accordingly, InnoSer offers preclinical research services focusing on evaluating therapeutic efficacy in the Fmr1 KO mouse model via multiple validated behavioral readouts. 

✓  Model is characterized on the C57BL/6J and FVB background. 

✓  Altered spontaneous behaviours, cognitive impairment, hyperactivity, altered anxiety levels, and decreases in social interactions.

✓  Changes in Event-Related Potentials (ERP) in electrocorticography (ECoG) typically observed in FXS patients are detectable in Fmr1 KO mice.

✓  Phenotypic similarities observed are confirmed by extensive in-house validation via several protocols using automated home-cage systems (PhenoTyper™) and conventional behavioural tests (portion of results published together with Kramvis et al., 2013).

✓  Fmr1 KO mice show key FXS characteristics at young age (starting at 6 weeks of age), allowing efficacy testing of targeted interventions as early as post-natal day 1.

European based preclinical CRO offering Fragile X syndrome mouse models for drug development

Take advantage of InnoSer’s expertise, flexibility and collaborative approach for your research. We support our clients in identifying new drugs or applications, characterizing their pharmacological properties, and conducting safety and efficacy testing with state-of-the-art readout capabilities and histopathological analysis. 

InnoSer’s neurology expert team possesses relevant experience in working with multiple therapy types ranging from small molecules, peptides, enzymes, oligonucleotides, and gene therapy (viral vectors – e.g.. AAVs). 

Your Fragile X Syndrome Research Starts Here.

Access detailed study timelines, essential readouts, and robust validation data for the Fmr1 KO mouse model (on C57BL/6J and FVB backgrounds).

Fragile X syndrome (Fmr1) lealfet for download with sample data

Fmr1 KO Sample Data

Fmr1 KO Readouts

Key Behavioral Readouts in the Fmr1 KO Mouse Model

Biological Readouts

EEG analyses and post-mortem analyses:
 

Our Team’s Featured Publications

 

  • Kramvis, I., Mansvelder, H. D., Loos, M., & Meredith, R. (2013). Hyperactivity, perseveration and increased responding during attentional rule acquisition in the Fragile X mouse model. Frontiers in behavioral neuroscience, 7, 172. https://doi.org/10.3389/fnbeh.2013.00172

The People Behind Your Research

Thomas Vogels, PhD Neurology study director InnoSer

Thomas Vogels, PhD, In Vivo Neurology Study Director

Leads an expert team of scientists with vast experience in our Neurology models to help you choose the right model and guide your optimal study design. We provide the solution to accelerating your drug development.

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