Thermo Fisher Scientific CLN3 Polyclonal Antibody

Applications

• Western Blot (WB): 1:500–1:3,000

Product Specifications

Product Specific Information

• Recommended positive controls: Jurkat, Raji, NCI-H929, NT2D1
• Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.

Target Information

CLN3 is a highly glycosylated, hydrophobic, 438-amino acid protein with six transmembrane domains. The CLN3 protein localizes to the lysosomal membrane and plays a role in lysosomal function. It may act as a chaperone involved in the folding and unfolding of other proteins, such as 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). Symptoms include progressive loss of vision, seizures, and psychomotor disturbances.

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

제품 이미지

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