Vanishing White Matter (Eif2b)
Test novel vanishing white matter disease therapeutics with relevant models possessing mutations in genes encoding the eukaryotic translation initiation factor 2B (eIF2B)
VWM can be studied using mouse models that have a genetic mutation in the same gene (eIF2B) that causes VWM in humans.
InnoSer offers unique services with an Eif2b4 / Eif2b5 mouse model of VWM disease through a collaboration with the Amsterdam Leukodystrophy Center at Amsterdam UMC. This model has been extensively characterized (Dooves et al. 2016). Proof of concept intervention studies in this model have identified a deregulated integrated stress response as a clear therapeutic target (Abbink et al. 2019).
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.
Eif2b4 / Eif2b5 mouse key model characteristics:
- VWM mice have homozygous point mutations in Eif2b4 or Eif2b5.
- VWM mice recapitulate motor symptoms that mimic those seen in humans with the disease, such as coordination problems. and tremors.
- VWM mice recapitulate cellular dysfunction seen in patients (e.g., deregulated ISR, astrocyte dysfunction).
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Key readouts
Test the efficacy of your treatments in the following battery of behavioural tests:
- Spontaneous Behavior
- Balance beam
- CatWalk gait analysis
- Neurological scoring
- Histology (e.g., nestin/GFAP, S100B)
- RT-qPCR (ISR markers)
- Western blot (MBP)
Eif2b5 mutant mice show impairments in the Balance Beam test
The Balance Beam sensorimotor coordination test scores the ability of mice to traverse a stationary horizontal rod. Coordination of the mouse is measured by the latency to cross the beam and the number of foot slips made.
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AAALAC Accreditation
InnoSer has earned the AAALAC accreditation, demonstrating our commitment to responsible animal care and use. AAALAC International is a nonprofit organization that promotes the humane treatment of animals in science through voluntary accreditation and assessment programs. Our accreditation is valid for three years, incl. 2023. Read more about the AAALAC accreditation programme here.
Animal Welfare
The 3Rs impact everything from policy and regulatory change to the development and uptake of new technologies and approaches. This is why InnoSer has ongoing commitment and monitoring of these processes. The steps we practice maximize our ability to replace, reduce and refine animal involvement and facilitate our commitment to these principles when it comes to research and drug development.
References
- Dooves S, Bugiani M, Postma NL, Polder E, Land N, Horan ST, van Deijk AL, van de Kreeke A, Jacobs G, Vuong C, Klooster J. Astrocytes are central in the pathomechanisms of vanishing white matter. The Journal of clinical investigation. 2016 Apr 1;126(4):1512-24.
- Abbink TE, Wisse LE, Jaku E, Thiecke MJ, Voltolini‐González D, Fritsen H, Bobeldijk S, Ter Braak TJ, Polder E, Postma NL, Bugiani M. Vanishing white matter: deregulated integrated stress response as therapy target. Annals of clinical and translational neurology. 2019 Aug;6(8):1407-22.
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