Neurology Models

Naason Science offers tools to study CNS Diseases in rodents and larger animals. From in vitro systems to large and small animal in vivo models Naason Science provides a tool box of state-of-the-art technologies that allow for rapid and effective testing of preclinical drug candidates.

Naason Science scientists have expertise in all major CNS diseases and tools to develop novel therapies for rare and orphan diseases.


R6/2 Transgenic Mouse Model of Huntington´s Disease

R6/2 mouse, which expresses mutant human exon 1 HTT was the first genetically modified mouse line  produced and has been the most extensively studied and utilized mouse model of HD.​

Naason Science has completed validation of R6/2 mouse line for the study of HD; the model paradigm has a complete functional and behavioral battery, imaging (MRI/MRS) and histological and molecular analysis of various regions of brain. 

Naason Science model assays:

  • ​Grip Strength
  • ​RotaRod
  • Home cage rearing
  • Home cage locomotor activity
  • Imaging (MRI/MRS, PET-SPECT / CT)
  • ​Histology / Stereology

Naason´s MDX Model of Duchenne Muscular Dystrophy

MDX mouse (on a C57BL/10ScSn-Dmdmdx/J) is a the most highly used model for the study of DMD.

Naason Science has completed validation of it’s MDX mouse model for the study of DMD, the model paradigm has a complete functional and behavioral battery as well as imaging (MRI) and histological analysis of limb muscles, diaphragm and heart. In addition, gene expression rounds out the paradigm.

BACHD Transgenic Rat Model

BACHD rat line is Huntington’s Disease transgenic rat model using human bacterial artificial chromosome (BAC) which contains the full-length HTT genomic sequence with 97 CAG/CAA repeats. BACHD rats display a robust, early-onset and progressive HD-like phenotype including motor deficits and anxiety-related symptoms. The phenotype is characterized by striatal dysfunction, followed by progressive brain volume reduction starting in the striatum. Neuropathologically, the distribution of neuropil aggregates and nuclear accumulations of N-terminal mutant huntingtin in BACHD rats is similar to that seen in HD patients. ​ 

Brain volume reduction.

Brain volume reduction.

GNAL Isoform 2-knockout Rat Model

Gnal KD/KO rat line is a model for an autosomal dominant neurological disorder called Dystonia 25. Dystonia-25 is characterized by adult onset of focal dystonia, usually involving the neck, the face and laryngeal muscles. Gnal KD/KO line was created by targeting the first exon of Gnal isoform 2 in rats using CRISPR/Caspase-9 system. The model displays reduced locomotor activities and impaired rotarod performance as well as impaired corticostriatal LTD. ​​ 

Increased surface expression of AMPAR in Gnal+/- striatal neurons compared to WT after treatment.

Increased surface expression of AMPAR in Gnal+/- striatal neurons compared to WT after treatment.


CNS therapeutic areas and models we are currently working with:

  • ​​Huntington Disease
  • Parkinson´s Disease
    ​- A-synuclein, 6-OHDA​
  • Alzheimer´s Disease
    ​- Tg2576 Mouse​
  • Multiple Sclerosis​
    ​- EAE, PLP & Cupriz Models​
  • ​ALS
    ​- SOD1 Mouse​
  • ​​Stroke
    ​- pMCAO, tMCAO
  • Ischemia
  • ​Muscular Dystrophy
  • Batten 
    ​- CLN3 Mouse​
  • Other models
    - SCA 3 & dystonia​
    ​- BT/BT Mouse​​