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Proteintech
CLN3 Monoclonal Antibody (1E10A9)
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Product Details
67957-1-IG150UL
Product Specifications
Species Reactivity
Human
Host/Isotype
Mouse
/ IgG1
Class
Monoclonal
Type
Antibody
Clone
1E10A9
Conjugate
Form
Liquid
Concentration
1 mg/mL
Purification
Protein G
Storage buffer
PBS, pH 7.3, with 50% glycerol
Contains
0.02% sodium azide
Storage conditions
-20°C
Shipping conditions
Wet ice
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: Batten disease protein; Battenin; ceroid-lipofuscinosis, neuronal 3; CLN 3; MGC102840; Protein CLN3
Gene Aliases: BTS; CLN3; JNCL
UniProt ID: (Human) Q13286
Entrez Gene ID: (Human) 1201
action potential
globoside metabolic process
cellular amino acid metabolic process
ceramide metabolic process
glucosylceramide metabolic process
galactosylceramide metabolic process
sphingomyelin metabolic process
receptor-mediated endocytosis
vacuolar transport
lysosome organization
lysosomal lumen acidification
associative learning
arginine transport
macroautophagy
negative regulation of macroautophagy
protein processing
protein catabolic process
ionotropic glutamate receptor signaling pathway
lysosomal lumen pH elevation
neurotransmitter metabolic process
amyloid precursor protein catabolic process
negative regulation of apoptotic process
negative regulation of catalytic activity
negative regulation of neuron apoptotic process
negative regulation of proteolysis
vesicle transport along microtubule
neuromuscular process controlling balance
regulation of cytosolic calcium ion concentration
membrane organization
autophagosome maturation
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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