Sacsin Antibody / SACS (RQ4029)

Description

Sacsin, encoded by the SACS gene, is a large, multifunctional protein that plays a critical role in cytoskeletal dynamics, protein folding, and mitochondrial maintenance. It is highly expressed in the central nervous system, particularly in cerebellar Purkinje cells, where it contributes to neuronal integrity and intracellular transport. Sacsin contains multiple functional domains, including a ubiquitin-like domain, Hsp90-like chaperone motifs, and a HEPN domain, suggesting a role in protein quality control and stress response pathways.

Mutations in the SACS gene are the primary cause of autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), a neurodegenerative disorder characterized by early-onset ataxia, spasticity, and peripheral neuropathy. Loss of Sacsin function results in abnormal intermediate filament organization and mitochondrial dysfunction, highlighting its importance in neuronal homeostasis and long-term motor coordination.

The Sacsin antibody is a powerful reagent for investigating the molecular mechanisms underlying neurodegeneration and mitochondrial biology. With proven utility in western blot, immunohistochemistry, and immunofluorescence applications, the Sacsin antibody enables reliable detection of endogenous Sacsin in tissue and cell samples. Researchers studying ataxia, cytoskeletal regulation, or neuronal pathology rely on the Sacsin antibody for its specificity and consistent performance across experimental systems.

Application Notes

Optimal dilution of the Sacsin antibody should be determined by the researcher.

Immunogen

A recombinant human partial protein corresponding to amino acids E3709-L3909 was used as the immunogen for the Sacsin antibody.

Storage

After reconstitution, the Sacsin antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.