This table summarizes the differences between important mouse models for ALS to help you easily find the right strain for your research. Use this resource to identify and select the most appropriate mouse model of ALS for your research and to obtain a guide to help you efficiently work with these mice.

The most widely used mouse model of ALS is a based on expression of the human SOD1 protein containing the G93A mutation (Philips and Rothstein, 2015; Lutz, 2018). The mSOD1 model has been instrumental in describing putative cellular dysfunction during disease pathogenesis, such as the non-cell autonomous nature of ALS (Nagai et al., 2007 ...

2022年3月8日 · The diagram outlines the various amyotrophic lateral sclerosis (ALS)-like pathologies (red) and phenotypes (grey) that can be observed in mouse models, including neuronal death, pathological...

2024年6月7日 · Amyotrophic lateral sclerosis (ALS) is a debilitating motor neuron disease and lacks effective disease-modifying treatments. This study utilizes a comprehensive...

2018年8月15日 · The genetic discovery in ALS comes at a time when genetic engineering technologies in mice are highly efficient through CRISPR/Cas9 and can be applied to a wide array of genetic backgrounds. New mouse resources in the forms of the Collaborative Cross and Diversity Outbred panels provide us with unique opportunities to study these mutations on ...

Research mouse models of ALS disease with motor dysfunction, cytoplasmic aggregates, neurodegeneration, activated microglia & neuromuscular junction pathology. Cytoplasmic TDP-43 (TDP43; TARDBP) aggregates are a hallmark of familial and sporadic Amyotrophic Lateral Sclerosis (ALS).

2019年7月22日 · Here we show that ALS-prone Sod1 transgenic (Sod1 -Tg) mice have a pre-symptomatic, vivarium-dependent dysbiosis and altered metabolite configuration, coupled with an exacerbated disease under...

By organizing information related to the phenotypic characterization of selected ALS models, this resource conveys what is known about each one and facilitates comparison between models. The curation of ALS models was supported in part by a grant from the NINDS. At 6 months, neuron loss observed in cortex, including layer V of motor cortex.

2017年12月1日 · Here, we review the mouse models that are key for drug development in ALS. The key features of each genetic subgroup are discussed and the models are compared. We also propose how the models could be further developed to better model ALS and thus more effectively advance ALS drug discovery.

Cytoplasmic TDP-43 (or TDP43) aggregates are a hallmark of familial and sporadic ALS. While several transgenic (tg) mouse models of amyotrophic lateral sclerosis (ALS; also called motor neuron disease [MND]) with TDP-43 aggregation exist, they each have their respective strengths and weaknesses.