Neuromuscular Junction Disorders
Animal models are essential for preclinical studies of neuromuscular junction (NMJ) disorders. Our company offers a range of animal models for studying the precise organization and function of the NMJ, providing important tools for exploring disease mechanisms and guiding appropriate therapy. Our team of experts can work with you to design and implement custom disease models and preclinical studies, which will help you develop safe and effective NMJ disease therapy methods.
Background
NMJ disorders are a group of conditions that affect signal transmission between nerves and muscles. Clinical manifestations include muscle weakness, paralysis, and other symptoms. There are many disorders associated with NMJ dysfunction and almost 90% of neuromuscular disorders (NMD) are classified as rare diseases. These disorders include myasthenia gravis (MG), congenital myasthenic syndrome (CMS), Lambert‑Eaton myasthenic syndrome (LEMS), and spinal muscular atrophy (SMA).
Animal models have been widely used to study NMJ mechanisms and to develop new therapies, including C. elegans, Drosophila, and zebrafish. Among them, rodents have become the most commonly used model due to their large size and easy access, providing most of the data for current preclinical studies. In addition, advances in human induced pluripotent stem cell (iPSC) technology provide new opportunities to study NMJ.
Fig. 1 Schematic diagram of the neuromuscular synaptic release of neurotransmitters. (Yan L, et al., 2022)
Our Services
We offer a range of disease modeling services that can help researchers develop and study animal models of neuromuscular junction disorders. These services include genetic engineering of animal models, generation of patient-derived xenografts, and in vitro analysis of disease models. We customize animal models with state-of-the-art gene editing techniques to express specific genes or mutations associated with NMJ diseases. Moreover, we comprehensively study multiple aspects of the phenotype, such as analysis of functional, biochemical, and structural features, to help our clients obtain the maximum information from these models. Our animal models include, but are not limited to:
- Rodent models of experimental autoimmune myasthenia gravis (EAMG)
We have produced EAMG models by administering autoantigens and generated models for LRP4, MuSK, and AChR. We typically produce EAMG models by immunizing the mice or rats with AChR in complete Freund's adjuvant (CFA). We offer the following AChR‑related EAMG models: - Mouse models: C57Bl/6J, HLA‑DQ8 transgenic B10 B6
- Rat models: HLA‑DQ8 transgenic B10, Lewis rats
- Canine models of spontaneous acquired autoimmune myasthenia gravis
We offer our clients canine models of spontaneously acquired autoimmune AChR antibody-positive myasthenia gravis. This natural model has many similarities to human disease, including pathogenetic features, diagnostic similarities (AChR autoantibodies), and co-morbidity.
- Rabbit models of Guillain–Barré syndrome (GBS)
We help our clients generate rabbit neuropathy models based on ganglioside immunization to study GBS.
- Rodent models of LEMS
We passively transfer LEMS to rodents by chronic injection of plasma, serum, or purified IgG to replicate the hallmark electrophysiological symptoms of LEMS. And we provide our clients with direct immune models, which are useful for characterizing the precise antigen-antibody interactions responsible for the autoimmune response.
Our company has over 20 years of experience in animal model development and use in preclinical research. One of the advantages of working with our company is our commitment to quality. We use rigorous quality control processes to ensure that our disease models are accurate and reliable. We also work closely with our clients to ensure that their research needs are met and they receive the highest level of customer service. If you are interested in our disease modeling services, please contact us for more information.
Reference
- Yan, L.; et al. Neuromuscular junction disorders: Experimental models and pathophysiological mechanisms. Acta Neurobiol Exp, 2022, 82: 501-510.
All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.