CLN3 is a highly glycosylated, hydrophobic, 438-amino acid protein with 6 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, namely subunit C of the ATP synthase complex.
Mutations in the CLN3 gene cause Batten disease, a recessively inherited neurodegenerative disorder of childhood caused by lysosomal accumulation of hydrophobic material, mainly ATP synthase subunit C.
Batten disease is the most common form of a group of disorders known as neuronal ceroid lipofuscinoses (NCLs).
Symptoms of Batten disease include progressive loss of vision, seizures, and psychomotor disturbances.
Applications:Suitable for use in Western Blot, Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid PBS, 0.1% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of CLN3. Species Crossreactivity: Human, mouse, rat
