CLN3 Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech
Rabbit Polyclonal Antibody
Manufacturer: Proteintech Group Inc 203861AP
DescriptionCLN3 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.
|PBS with 50% glycerol and 0.02% sodium azide; pH 7.3|
|Batten disease protein, BATTENIN, BTS, CLN3, JNCL, Protein CLN3|
|1201, 12752, 293485|
|Immunocytochemistry, Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot|
|CLN3 Fusion Protein Ag14224|
|Antigen Affinity Chromatography|
|Human, Mouse, Rat|
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