Amyotrophic Lateral Sclerosis (ALS) Model

Sichuan Greentech Bioscience provides a range of animal models for neurodegenerative diseases, including Parkinson’s disease (PD), Alzheimer’s disease (AD), and amyotrophic lateral sclerosis (ALS), to support preclinical efficacy evaluation of therapeutic candidates for common neurodegenerative disorders.

Our ALS Model: hSOD1-G93A Transgenic Mouse Model

Transgenic mouse models carrying disease-associated mutations play an important role in ALS research. Although familial ALS involves multiple genetic mechanisms, these transgenic models exhibit similar ALS-related phenotypes (Figure 1). SOD1 was the first gene identified as being associated with fALS. The hSOD1-G93A transgenic mouse is one of the most widely used ALS animal models and exhibits motor neuron loss, paralysis, and premature death. The FDA-approved ALS therapies riluzole and edaravone have both been evaluated using the hSOD1-G93A mouse model.

Figure 1. ALS-like pathology (red) and ALS-like phenotypes (gray) in ALS mouse models. Some models also exhibit frontotemporal dementia (FTD)-like features (blue) (Todd & Petrucelli, 2022). Although ALS and FTD affect different neurons, clinical observations indicate that they frequently occur in the same patient.

Representative Case Studies