Thermo Fisher Scientific CLN3 Polyclonal Antibody

Applications and Tested Dilution

Product Specifications

Product Specific Information

The antibody was affinity-purified from rabbit antiserum by affinity chromatography using an epitope-specific immunogen. The purity is greater than 95% as determined by SDS-PAGE.

Target Information

CLN3 is a highly glycosylated, hydrophobic, 438-amino acid protein with six transmembrane domains. It localizes to the lysosomal membrane and plays a role in lysosomal function. CLN3 may act as a chaperone involved in the folding and unfolding of other proteins, particularly subunit C of the ATP synthase complex.
Mutations in the CLN3 gene cause Batten disease, a recessively inherited neurodegenerative disorder of childhood characterized by lysosomal accumulation of hydrophobic material, mainly ATP synthase subunit C. Batten disease is the most common form of neuronal ceroid lipofuscinoses (NCLs) and is associated with progressive loss of vision, seizures, and psychomotor disturbances.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.