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Animal Models for ALS Disease

Animal Models for Neuromuscular Rare Diseases

Our company is committed to providing researchers with a variety of experimental animal models for simulating Amyotrophic Lateral Sclerosis (ALS) disease and studying the underlying mechanisms of the disease in a controlled environment. By understanding these mechanisms, researchers can identify potential drug targets and test new therapies in preclinical studies. Our team of experts has years of experience in disease modeling and preclinical research and uses state-of-the-art equipment and techniques to help our clients achieve their research goals.

Background

ALS is a neurodegenerative disease that affects motor neurons in the brain and spinal cord. It is a progressive disease that leads to muscle weakness, paralysis, and eventually death. The symptoms of ALS vary widely among patients. Two widespread forms of ALS have been described, including familial ALS (fALS) and sporadic ALS (sALS), the latter accounting for 90% of cases.

ALS has a complex molecular basis and pathological mechanisms. Animal models may help researchers understand the disease's pathological process and develop new therapies. Several important models have been generated to study ALS including nematodes, flies, mice, guinea pigs, dogs, and others. Each of these models offers distinct advantages for ALS research. Currently, with the discovery of an increasing number of genetic mutations in ALS and the development of gene editing technologies, new animal model systems are being developed to facilitate the development of new therapies. 

Fig. 1 Drug target validation in mouse models of ALS.

Fig. 1 Drug target validation in mouse models of ALS. (Paratore S, et al., 2012)

Disease Modeling Services

  • Generation of zebrafish (Danio rerio) models
    Zebrafish are a valuable model for studying neurodegenerative diseases such as ALS. They offer unique advantages such as ease of genetic modification, high conservation of disease-associated genes, and efficiency of toxin screening. Using the optical transparency of the embryonic stage, we insert ALS gene mutations into zebrafish to study their effects on motor neurons. We generate environmental and genetic zebrafish models for our clients, including:
    • Bisphenol A (BPA) exposure in zebrafish
    • Zebrafish carrying RNA-binding protein fused in sarcoma (FUS) mutations
    • C9orf72 knockdown zebrafish model
    • G93R-mSOD1 zebrafish model
    • TDP43-A315T zebrafish model
  • Generation of rodent models
    Rodent models are the most widely used animal models in ALS research. Because of their complex nervous systems, they provide important tools for testing potential therapies for ALS such as gene therapy and stem cell approaches. As new mutations are identified, our researchers are committed to developing more mouse models to provide scientists with a variety of pathogenic backgrounds. Rodent models we offer include:
    • Rodents carrying FUS mutations
    • FVB-C9orf72 BAC mouse model
    • Cu/Zn superoxide dismutase 1 (SOD1)-G93A mouse model
    • TDP43-Q331K mouse model
    • Rodent models carrying rare mutations linked to ALS, such as rodents carrying ALS2/SEXT/OPTN/UBQLN2/VCP mutations
    • Non-genetic rodent models, such as guinea pig ALS models and L-BMAA-induced ALS rodent models
  • Generation of Drosophila Melanogaster models
    Drosophila melanogaster is widely used to study ALS due to its complete genome sequence, ease of handling, short life cycle, and cost-effectiveness. Its UAS/Gal4 system is widely used to overexpress genes, including those associated with human diseases, making it a powerful genetic model. We offer several Drosophila melanogaster models carrying different ALS-associated mutations to our customers.
    • Drosophila melanogaster carrying SOD1 mutations
    • Drosophila melanogaster carrying TDP-43A315T mutations
    • Drosophila melanogaster carrying FUS mutations
    • Drosophila melanogaster carrying C9orf72 mutations
  • Other Animal Models
    We also provide our clients with other animal models for translational research on this dramatic disease, including Caenorhabditis elegans models, canine models, swine models, and non-human primate models.

As a leading provider of disease modeling and preclinical research services, our company has several strengths in ALS research. We use technologies such as CRISPR/Cas9 to help our clients design and generate the most appropriate experimental animal models for preclinical ALS research. Our ALS animal models cover a wide range of animals, including vertebrates and invertebrates. If you are interested in our disease modeling services, please contact us for more information.

Reference

  • Paratore, S.; et al. Identification of pharmacological targets in amyotrophic lateral sclerosis through genomic analysis of deregulated genes and pathways. Current Genomics, 2012, 13(4): 321-333.

All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.

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