Additionally, because autopsy tissue is obtained at dis- ease end stage and after a period of post-mortem ischemia, it is difficult to determine if the changes observed only occur late in disease progression. While a variety of peripherally derived inflammatory cell types have also been observed ...
The extensive pathological alterations in L2/3 neurons in both motor and frontal cortices could thus contribute to the cognitive and behavioral symptoms in C9-ALS81. Whereas genes downregulated in C9-ALS excitatory neurons showed the most significant enrichments for categories associated with neuron-...
In these preceding experiments our gRNAs were expressed without the need for CasRx to mature a pre-gRNA prior to target recognition and binding. As the gRNA in a therapeutic vector would be in a pre-gRNA configuration, we next investigated whether CasRx could mature a pre-gRNA and maintain ta...
In these preceding experiments our gRNAs were expressed without the need for CasRx to mature a pre-gRNA prior to target recognition and binding. As the gRNA in a therapeutic vector would be in a pre-gRNA configuration, we next investigated whether CasRx could mature a pre-gRNA and maintain ta...
The tissue slide was placed on the stage of an inverted microscope. A custom gasket was then clamped onto the slide, allowing the tissue to be submerged in 1.5 mL of buffer solution. The microcapillary tip is connected to a syringe pump primed with buffer solution, allowing for accurate ...
To gain insight into the transcriptomic changes taking place during disease progression this study was performed at three stages: asymptomatic, symptomatic and end stage. We showed that before appearance of motor symptoms, the major disrupted mechanisms were linked with the immune system/inflammatory ...
Although few juvenile cases have been reported, ALS has generally an adult onset and progresses rather rapidly in most patients (few years from onset to end-stage). There are two forms of ALS: sporadic (sALS) and familiar (fALS). About 5% of ALS cases are inherited, in which there is ...
Hospice is a process to end-of-life care and a kind of support facility for terminally ill patients. It provides comforting care, patient-centered care and related services. Comforting care relieves discomfort without improving the patient’s condition or curing his illness. Hospice is extended in...
Whereas postmortem material can be regarded as the end-stage of the disease mechanism, in vitro models with a disease-relevant phenotype might be able to provide earlier mechanistic insights. Additionally, various knockdown approaches (i.e. siRNA, antisense oligonucleotides, CRISPR/Cas9) can be harne...
At these stages, the animals showed symptoms of ALS (mutSOD1 and mutTDP43) and FTD (mutC9ORF72). Only males were used for these experiments; since we have not analyzed female mice, we do not know whether the sex of the animals impacts the findings. For the in vivo experiments in ...