Development of ALS Modeling Viruses

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that selectively invades upper and lower motor neurons, causing progressive paralysis and muscle atrophy. Its pathogenesis is unknown. It is characterized by progressively worsening limb weakness and muscle atrophy combined with pyramidal signs, accompanied by symptoms of medullary involvement such as slurred speech and dysphagia, and respiratory muscle involvement, which can lead to dyspnoea, nocturnal sleep apnoea, and ultimately respiratory failure. To further your research on this disease, Alfa Cytology provides you with animal models of ALS, including transgenic model animals and AAV vectors, as well as viral products for modeling.

We provide ALS modeling services and modeling-related viral products

ALS transgenic modeling

We offer several different ALS transgenic model mice including TDP-43 / TDP-25 / TDP-43M337V, SOD1, and the GGGGCC repeat in the non-coding region of C9ORF72 (chromosome 9 open reading frame 72 gene). TDP-43 wild-type and its truncations and mutants are the most established animal models for studying the pathogenesis of ALS.

AAV-induced ALS models

We were able to mimic some of the symptoms and pathological features of ALS safely and feasibly by expressing TDP-43 via temporal vein injection and stereotactic injection with the help of AAV vectors.

ALS modelling-related products

In terms of how animal models are constructed, neuronal death and motor deficits caused by overexpression of ALS-associated genes are more pronounced, and can be used to simulate middle to late-stage disease in patients. Knock-in genes have a lesser effect on mice and are generally considered to simulate early to mid-stage disease.

The pathogenesis of ALS is currently unclear, and possible mechanisms include excitatory amino acid toxicity, oxidative stress, abnormal mitochondrial function, abnormal phosphorylation of neurofilament proteins, apoptosis, and inflammatory cascade responses. Alfa Cytology also offers a range of gene-edited mouse models, as well as custom or collaborative development of gene-edited mouse models in response to researchers' needs. These include knockout, knock-in, point mutation, humanized mouse models, and surgical disease models in mice and rats to accelerate ALS pharmacodynamic validation experiments. Please contact us for more technical solutions.