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 strond neuroinflammation in the cortex and hippocampus, and thus exhibiting the neuronopathic phenotype 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.
QPS Austria is also ready to provide samples (brain tissue, CSF etc.) from these animals for analyses in your laboratory.
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