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)

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Vanishing White Matter (VWM) is clinically characterized by progressive motor dysfunction and mild cognitive decline.  VWM is caused by 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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Belgian based preclinical neurology CRO mouse models

Key readouts

Test the efficacy of your treatments in the following battery of behavioural tests:

Test the efficacy of your treatments with the following biological readouts:

  • Histology (e.g., nestin/GFAP, S100B) 
  • RT-qPCR (ISR markers) 
  • Western blot (MBP)

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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.

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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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