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Invitrogen
CLN3 Polyclonal Antibody
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Please note: We are reviewing Western blot images included in the antibody testing data in our catalog, including those provided by third parties. Unless expressly labeled or annotated as “raw-unedited”, Western blot images included in the antibody testing data in our catalog may have been edited, optimized or otherwise adjusted for presentation.
Product Details
PA5-64103
Product Specifications
Species Reactivity
Human
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
Recombinant protein corresponding to Human CLN3. Recombinant protein control fragment (Product #RP-104248).
Conjugate
Unconjugated
Unconjugated
Unconjugated
Form
Liquid
Concentration
0.1 mg/mL
Amount
10 µg
Purification
Antigen affinity chromatography
Storage buffer
PBS, pH 7.2, with 40% glycerol
Contains
0.02% sodium azide
Storage conditions
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Shipping conditions
Ambient (domestic); Wet ice (international)
RRID
Product Specific Information
Immunogen sequence: EEEAESAARQ PLIRTEAPES KPGSSSSLSL RERWTVF
Highest antigen sequence identity to the following orthologs: Mouse - 78%, Rat - 73%.
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; batten disease protein; ceroid-lipofuscinosis, neuronal 3; CLN 3; CLN3, battenin; MGC102840; unnamed protein product
Gene Aliases: BTN1; BTS; JNCL; RP101; SLC29B1
UniProt ID: (Human) Q13286
Entrez Gene ID: (Human) 1201
action potential
amino acid transport
endocytosis
receptor-mediated endocytosis
lysosome organization
lysosomal lumen acidification
learning or memory
associative learning
cellular water homeostasis
regulation of autophagy
regulation of fibroblast migration
regulation of autophagosome size
protein processing
cellular cation homeostasis
regulation of proteolysis
regulation of synaptic transmission, GABAergic
ionotropic glutamate receptor signaling pathway
ceramide transport
lysosomal lumen pH elevation
renal potassium excretion
amyloid precursor protein catabolic process
positive regulation of Golgi to plasma membrane protein transport
negative regulation of apoptotic process
negative regulation of neuron apoptotic process
plasma membrane raft organization
negative regulation of proteolysis
glycerophospholipid biosynthetic process
glycolipid transport
vesicle transport along microtubule
regulation of short-term neuronal synaptic plasticity
positive regulation of pinocytosis
neuromuscular process controlling balance
regulation of cytosolic calcium ion concentration
regulation of cytoskeleton organization
regulation of synaptic transmission, glutamatergic
membrane organization
autophagosome-lysosome fusion
regulation of protein processing
Golgi to lysosome transport
phagosome-lysosome fusion
autophagosome maturation
regulation of cellular response to osmotic stress
regulation of arginine biosynthetic process
regulation of autophagosome maturation
regulation of protein localization to plasma membrane
arginine transmembrane transport
lysosomal protein catabolic process
regulation of phagosome maturation
regulation of modification of synaptic structure
positive regulation of caveolin-mediated endocytosis
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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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