Measurement of ultrasonic vocalizations (USV) provides a unique and gentle method to study social behavior in rodents. Mice emit ultrasonic vocalizations in different social contexts throughout their lifespan, including pups when separated from the dam and adult males when exposed to a sexually receptive female. In the latter context, male mice typically emit ultrasonic vocalizations … Read more
Spontaneous neuronal activity of cultivated cells is one of the physiologically most relevant read-outs related to neuronal health. Calcium concentration is widely used in neuroscience as a competent indirect reporter of neural activity, as action potential (AP) firing triggers large influxes of Ca2+ through voltage-gated calcium channels. Utilizing IncuCyte® NeuroBurst, a genetically-encoded calcium indicator (GECI), in combination … Read more
Compound muscle action potential (CMAP) is an electrophysiological technique commonly employed to monitor the functional status of muscles in anesthetized animals. CMAP measurements offer insight into physiological development as well as degeneration of the peripheral neuromuscular system. As part of the electromyographic (EMG) evaluation, CMAP is regularly used in diagnostic and clinical human studies, providing … Read more
A mutation of the FMR1 gene (Fragile X mental retardation 1) leads to a X-linked genetic disorder called Fragile X syndrome (FXS), the most frequently inherited form of intellectual disability and the monogenic cause of autism spectrum disorder (ASD). Patients of ASD present communication and socialization impairments, repetitive behaviors with restricted interest, and hyperactivity. Silencing … Read more
Huntington’s disease (HD) is an autosomal-dominantly inherited, fatal, neurodegenerative disorder. Patients present with motor dysfunctions, psychiatric symptoms, cognitive impairments, and metabolic abnormalities. HD is solely caused by the expansion of an unstable repeat of CAG base triplets in the coding region of the Huntingtin gene, HTT. The age of disease onset correlates inversely with the … Read more
After validating muscle weakness and motor deficits in young 6neo mice as model of Pompe disease, our team now evaluated acid α-glucosidase (GAA) enzyme activity to validate enzyme reduction and glycogen substrate levels in non-neuronal and neuronal tissue of this lysosomal storage disease mouse model. Quadriceps femoris muscle and cortical tissue of Pompe 6neo mice … Read more
Scientific studies suggest that the leukotriene signaling pathway, an inflammatory pathway originally known for its role in asthma, is involved in the pathogenesis of various neurodegenerative diseases including Parkinson’s disease (PD). We evaluated whether blocking of leukotriene signaling using the approved anti-asthmatic drug Montelukast (MTK) improves motor impairments in the Line 61 mouse model of … Read more
Mutations in the human GBA (glucosylceramidase-β) gene are associated with lowered glucosylceramidase-β (GCase) activity and are shown to be relevant for the development of Gaucher disease. The enzyme is further highly discussed as therapeutic target in Parkinson`s disease research. The GBA-D409V-KI mouse model expresses the mutant D427V GBA protein which corresponds to the D409V mutation … Read more
The importance of microglia for neurodegenerative diseases is well-known and these cells are therefore frequently used as a target for new pharmacological interventions. To study this cell type, isolation of early postnatal microglia from mice is a great tool but does not properly reflect conditions in aged or diseased individuals. Isolation of viable microglia from … Read more
Multi-Channel Imaging to Visualize Renal Inflammation in an Alport Disease Mouse Model While we are specialists for neuronal tissue, we also routinely process tissue of peripheral organs: Kidney of the Alport syndrome mouse model Col4A3-/- was labeled for macrophages and leukocytes (Fig.1) as well as T-lymphocytes and macrophages (Fig.2), followed by automated slide scanning. Figure … Read more
Establishment of the CCl4 mouse model allows preclinical testing of your new anti-fibrotic drugs. Liver fibrosis occurs in most types of chronic liver diseases. In humans, researchers were already able to show that liver fibrosis is reversible, fostering the development of anti-fibrotic drugs. To test the efficacy of these new drugs, proper preclinical models are … Read more
Newest results of our R&D team at Scantox validate severe and very early motor deficits in hA53Ttg mice, making this model well-suited for preclinical Parkinson’s disease (PD) research. PD belongs to the most devastating neurodegenerative diseases of the 21st century. The development of new drugs halting disease progression is therefore the main focus of PD … Read more
