Superoxide dismutase Pre-formed Fibrils
Product Sizes
100 ug
SPR-470-100UG
2 x 100 ug
SPR-470-2X100UG
5 x 100 ug
SPR-470-5X100UG
About this Product
- SKU:
- SPR-470
- Additional Names:
- Superoxide dismutase 1, SOD1, ALS1, IPOA, Cu/Zn superoxide dismutase, Superoxide dismutase [Cu-Zn], hSOD1, SODC, Cytosolic superoxide dismutase, Erythrocuprein, Hemocuprein, Cytocuprein, Indophenoloxidase A, SOD1 PFFs
- Application:
- Cell-based/Functional Assay, SDS-PAGE, Western Blot
- Buffer:
- PB pH 7.4, 5 mM EDTA, 50 mM DTT
- CE/IVD:
- RUO
- Extra Details:
- Superoxide dismutase 1 (SOD1) is a copper/zinc-binding enzyme essential for cellular antioxidant defense, catalyzing the conversion of superoxide radicals into oxygen and hydrogen peroxide. Mutations or misfolding of SOD1 are strongly linked to amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder characterized by progressive motor neuron degeneration. In ALS, aberrant SOD1 conformations aggregate into toxic species, disrupting protein homeostasis and driving neuronal death. SOD1 Pre-formed Fibrils (PFFs) have emerged as powerful tools for modeling this pathogenic process. These synthetic fibrillar aggregates mimic the misfolded state of SOD1 observed in ALS and can seed the aggregation of endogenous SOD1 in vitro and in vivo. By initiating a cascade of protein misfolding, PFFs replicate key features of ALS pathology, including prion-like propagation and inclusion formation. This makes them invaluable for studying molecular mechanisms of aggregation, oxidative stress, and neurotoxicity. Researchers use SOD1 PFFs to develop robust cellular and animal models, enabling high-throughput screening of therapeutic candidates aimed at preventing or reversing aggregation. Beyond ALS, these models provide insights into shared pathways of protein misfolding implicated in other neurodegenerative diseases such as Alzheimer's and Parkinson's. By bridging mechanistic understanding and translational research, SOD1 PFFs accelerate the discovery of disease-modifying strategies for disorders driven by protein aggregation.
- Immunogen:
- SOD1 PFFs
- Molecular Weight:
- 15.936 kDa
- Purity:
- >95%
- Purification:
- Ion Exchange
- Shipping Conditions:
- Dry Ice
- Storage Conditions:
- -70[o]C
- Supplier:
- StressMarq Biosciences
- Type:
- Proteins, Peptides, Small Molecules & Other Biomolecules: Recombinant Proteins
- Manufacturer's Data Sheet:https://www.stressmarq.com/products/protein/sod-protein--spr-470/
