Histopathological phenotype of 6neo mice as model of Pompe disease
After validating in vivo muscle deficits, α-glucosidase deficits in the brain and muscle, as well as elevated glycogen levels in the brain and muscle, our R&D team now evaluated several disease-relevant biomarkers in Pompe 6neo mice longitudinally. In a first step, liver, heart, diaphragm, tibialis anterior and quadriceps tissue were evaluated by PAS staining to …
Repeated electromyographic measurements for longitudinal evaluation of muscle impairments
We are now able to offer longitudinal, repeated in vivo electromyographic measurements in rodent models with motor impairment. These analyses provide sensitive longitudinal readouts about your drug candidates that are unaffected by the environmental or emotional status of your animals. Typical readouts are the Compound Muscle Action Potential (CMAP), Motor Unit Action Potential (MUAP) and …
Translational read-out for preclinical Gaucher disease studies: Assessment of glucosylceramidase-β (GCase) activity in dried blood spots
Reduced GCase activity is a key causative hallmark of Gaucher disease. Monitoring changes in GCase activity at multiple time points from small volumes of whole blood, sampled as dried blood spots (DBS), can give valuable new insights on drug effects. Mutations in the human glucosylceramidase-β (GBA) gene and associated GCase activity are causative for Gaucher …
Repeated MPTP injections to model the Parkinson’s disease phenotype
Repeated MPTP injections into the mouse’ intraperitoneal space result in a Parkinson´s disease brain pathology shown as degeneration of dopaminergic neurons and neuroinflammation. Upon entering the brain, methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) oxidizes into the highly neurotoxic compound 1-methyl-4-phenylpyridinium (MPP+) which is selectively taken up by dopaminergic neurons. Inside neurons, MPP+ disrupts mitochondrial function and causes oxidative stress, …
Fluorescent In Situ Hybridization (FISH) to Analyze Gene Expression
Detection of mRNA by fluorescent in situ hybridization (FISH) is a great alternative for proteins that are hard to detect by immunofluorescence. FISH is also the method of choice for the analysis of expression efficacy during the development of new gene therapies. The Department of Histology of QPS Neuropharmacology therefore established the FISH labeling method …