4L/PS-NA transgenic Mouse Model

4L/PS-NA mice express low levels of prosaposin and saposins, as well as β-glucosidase (GCase) with a point mutation at position V394L/V394L and model Gaucher disease, the most common lysosomal storage disease. The neuronal disease variant is characterized by aggregated protein accumulations in the brain and associated neurological manifestations. Homozygous 4-24 weeks old 4L/PS-NA mice thus present with progressive α-synuclein aggregate accumulations in cortex, hippocampus, basal ganglia, brainstem and some cerebellar regions. Furthermore, 4L/PS-NA mice present a strong enlargement of leukocytes and macrophages in visceral organs like spleen, thymus, lung and liver as early as 5 weeks of age. With increasing age, 4L/PS-NA mice develop motor deficits and reduced muscle strength that is accompanied by strong neuroinflammation in the cortex and hippocampus, and thus exhibiting the neuronopathic phenotype of Gaucher disease.

4L/PS-NA Mice as Model of Gaucher Disease

Neuroinflammation and motor deficits of 5 to 18 weeks old 4L/PS-NA mice. A: Astrocytosis in the cortex of 4L/PS-NA mice as measured by GFAP immunoreactive area in percent, n=5; B: Latency to fall off the rod in seconds in the RotaRod test, n = 7. Mean+SEM. Two way ANOVA followed by Bonferroni multiple comparison test. *p<0.05; **p<0.01; ***p<0.01. *differences between genotypes; #differences between age groups;

QPS Austria is also ready to provide samples (brain tissue, CSF etc.) from these animals for analyses in your laboratory.

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