Ndufs4 Knockout Mice for Modeling Leigh Syndrome

Biomaterial Description

The Ndufs4 Knockout (KO) Mice are an innovative model for studying mitochondrial complex I deficiency, specifically designed to model the severe mitochondrial disease known as Leigh syndrome. These mice are genetically engineered to lack the Ndufs4 gene, which encodes a critical subunit of the NADH:ubiquinone oxidoreductase (Complex I) in the mitochondrial electron transport chain. Defects in mitochondrial complex I are the most prevalent among mitochondrial disorders. This deficiency leads to a range of neurometabolic symptoms, providing a robust model for studying mitochondrial disorders and their systemic effects.

Applications

Drug Development: Use these mice to screen and evaluate the efficacy of potential drugs targeting mitochondrial dysfunction and related neurodegenerative conditions.

Gene Therapy: Investigate gene therapy approaches to correct mitochondrial deficiencies and improve neurological outcomes.

Metabolic Studies: Explore the metabolic pathways affected by mitochondrial dysfunction and identify potential metabolic interventions.

Aging Studies: Study the impact of mitochondrial dysfunction on aging and test interventions that could extend healthy lifespan.

Advantages

Versatile Research Tool: These mice can be used to study mitochondrial dysfunction, neurodegenerative diseases, and metabolic disorders.

Therapeutic Development: Ideal for preclinical testing of novel therapeutic interventions aimed at mitigating mitochondrial diseases.

Conditional KO Model Available.

Distributor Information

Non-Exclusive License available.

Shipped from JAX:

JAX Stock #026963 – Conditional Cre Mice

JAX Stock #027058 – KO mice