Aging is a complex biological process characterized by a gradual decline in physiological functions and an increased susceptibility to diseases. Cellular senescence is a fundamental mechanism underlying aging, where cells irreversibly stop dividing in response to stress or damage, contributing to tissue dysfunction over time. While senescence has a protective role against tumorigenesis by preventing … Read more
After evaluating main phenotypic traits of Fmr1-KO mice showing increased activity, hyperactivity, anxiety, and repetitive behavior (Behavior Newsletter) we now evaluated brain pathological hallmarks of young Fmr1-KO mice. In Fmr1-KO mice, the Fmr1 gene on exon 5 is replaced by more than 200 CGG repeats with a neomycin resistance cassette. Animals thus present a valuable … Read more
In 2006, aggregates and mislocalization of the RNA binding protein TDP-43 were discovered in human amyotrophic lateral sclerosis (ALS) brain samples by Neumann and colleagues. Since then, in vitro and in vivo overexpression of TDP-43 were shown to cause ALS pathology and thus providing a suitable model to study underlying ALS mechanisms. However, transgenic ALS … Read more
Neuroinflammation, a complex immune response within the central nervous system, stands as both a shield and a sword in neurological health. It involves the activation of immune cells like microglia and astrocytes, the release of signaling molecules, and phagocytosis of pathogens. While it serves as a frontline defense against threats, dysregulation can fuel the progression … Read more
Injection of two different concentrations of rotenone directly into the right striatum of wild-type mice causes a significant increase of contralateral slips in the beam walk test when compared to the ipsilateral side of the same animals as well as the contralateral side of DMSO-injected littermates (Figure 1A). This phenotype is measurable for at least … Read more
B6.SOD1G93A transgenic mice, bred on a congenic C57BL/6 background, offer a compelling alternative to the commonly used SOD1(*G93A)1Gur mice. They exhibit a slightly slower progression of the amyotrophic lateral sclerosis (ALS)-specific phenotype compared to the original strain, which is conventionally bred on a mixed C57BL/6xSJL background. The slower disease course offers an extended window for … Read more
In a previous newsletter we already introduced the AAV2 hA53T-α-syn mouse model to present increased hA53T-α-syn levels in the unilaterally virus-injected substantia nigra and the caudate putamen of the same hemisphere compared to the contralateral hemisphere injected with control vector. Furthermore, we could show decreased tyrosine hydroxylase (TH) levels and increased Iba1 levels in the … Read more
We just established different patient-derived fibroblasts as models for various lysosomal storage diseases (LSDs), offering a valuable tool for drug screening. LSDs are a group of rare genetic disorders, characterized by deficiencies in specific enzymes that usually facilitate the lysosomal degradation process, leading to the accumulation of undegraded substances within the lysosome. These diseases can … Read more
We now offer advanced search strategy analysis of data gathered during Morris Water Maze (MWM) test without changes to the established protocol. The MWM has been a widely recognized method to analyze spatial navigation and learning for several decades. This makes it a valuable tool for the evaluation of cognitively impaired rodent models, used to … Read more
Newest results of our R&D team show highly increased total and phosphorylated tau (ptau) levels in transgenic PS19 mice already at early age. This rodent model for tauopathies expresses the T34 isoform and 4 microtubule binding repeats (1N4R) of the tau protein with P301S mutation under the regulatory control of the murine prion promoter (Prnp). … Read more
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 … Read more
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 … Read more