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Proteintech
CLN3 Polyclonal Antibody
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Product Details
20386-1-AP
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse,
Rat
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
CLN3 Fusion Protein Ag14224 (288-368 aa encoded by BC002394)
Conjugate
Form
Liquid
Concentration
0.25 mg/mL
Purification
Antigen affinity chromatography
Storage buffer
PBS, pH 7.3, with 50% glycerol
Contains
0.02% sodium azide
Storage conditions
-20°C
Shipping conditions
Wet ice (domestic); Dry ice (international)
Product Specific Information
Immunogen sequence: LVVVYFAEY FINQGLFELL FFWNTSLSHA QQYRWYQMLY QAGVFASRSS LRCCRIRFTW ALALLQCLNL VFLLADVWFG FL (288-368 aa encoded by BC002394)
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, juvenile (Batten, Spielmeyer-Vogt disease); ceroid-lipofuscinosis, neuronal 3; CLN 3; Cln3p; MGC102840; Protein CLN3
Gene Aliases: AI323623; BTS; CLN3; JNCL
UniProt ID: (Human) Q13286, (Mouse) Q61124
Entrez Gene ID: (Human) 1201, (Rat) 293485, (Mouse) 12752
action potential
globoside metabolic process
positive regulation of protein phosphorylation
cellular amino acid metabolic process
ceramide metabolic process
glucosylceramide metabolic process
galactosylceramide metabolic process
sphingomyelin metabolic process
amino acid transport
endocytosis
receptor-mediated endocytosis
vacuolar transport
lysosome organization
lysosomal lumen acidification
biological_process
associative learning
arginine transport
macroautophagy
negative regulation of macroautophagy
cell migration
protein processing
actin cytoskeleton organization
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
regulation of filopodium assembly
membrane organization
protein localization to membrane
protein localization to plasma membrane
autophagosome maturation
transport
regulation of gene expression
protein catabolic process
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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