hSOD1 Mouse Model
| Strain Name | hSOD1 Mouse Model |
|---|---|
| Strain Background | C57BL/6N |
Applications
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ALS gene therapy: Test anti-SOD1 ASOs, siRNA, or CRISPR knockdown for efficacy and safety.
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ALS pathophysiology: Study SOD1 misfolding, aggregation, and mitochondrial toxicity.
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Neuroinflammation & oxidative stress: Investigate glial activation and redox signaling.
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Preclinical PK/PD & tox: Use human SOD1 for antibody or oligonucleotide distribution analysis.
Strain Description
The hSOD1 Mouse Model carries a targeted knock-inof the human SOD1 gene. It replaces the murine Sod1 coding sequence at the endogenous locus. Importantly, it preserves native promoters and regulatory elements. This ensures physiological expressionof functional human Cu/Zn-superoxide dismutase in the same cell types where the endogenous gene is normally expressed, primarily motor neurons and hepatocytes. This human SOD1 knock-in mouse produces the authentic enzyme that functions in cellular antioxidant defense.
In this hSOD1 model, SOD1 catalyzes the dismutationof superoxide radicals into hydrogen peroxide and oxygen. It also regulates mitochondrial respiration and redox-sensitive signaling pathways. However, toxic misfoldingof mutant SOD1 triggers oxidative stress and motor neuron degeneration. For example, this process drives the hallmark pathologyof familial amyotrophic lateral sclerosis (ALS).
This humanized SOD1 model allows therapeutic agentsto bind human SOD1 in vivo. For instance, researchers can test anti-SOD1 antisense oligonucleotides (ASOs) or gene editors. Consequently, it provides a translational platform for evaluating SOD1-lowering strategies. It also supports studying mutant SOD1 aggregation and modeling human ALS pathologies that conventional models cannot replicate.
FAQ
Game-changing benefits？
While competitors highlight germline efficiency gains, shorter timelines and enhanced 3Rs animal welfare benefits for their technologies, these are merely incremental improvements over traditional approaches. In sharp contrast, our proprietary technology delivers fully pure, homogeneous lineages—every single cell of the mice is derived exclusively from totipotent ES cells, with guaranteed 100% germline transmission efficiency. To experience these unparalleled benefits firsthand, enquire about your custom mouse model project with us or order embryos for in-house validation at your facility.
How much for a project assessment?
•Free initial design proposal with zero obligations.
•Request a free quote!
Who owns the mouse lP? Do l need a licence from Mingceler?
All model generation projects of Mingceler operate under a fee-for-service framework.
IP Ownership: All intellectual property rights related to custom mouse models, including derived organs, tissues, cells, and biological materials, are the sole and exclusive property of the Client.
Third-Party Transfer Permission: The Client may independently decide to retain, utilize, or commercialize their custom models project materials (e.g., targeting vectors, ES cells, mouse lines) without the need for prior consent from Mingceler.
Licensing Exemption: The Client has full autonomy over all uses of the custom models or their derivatives, including but not limited to commercialization, distribution to third parties, and publication involving model data. No written license from Mingceler is required for such uses.