CLN3, Polyclonal Antibody

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

>=95% as determined by SDS-PAGE
Immunogen Affinity Purified

Liquid

PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-CLN3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

45,155 Da

CLN3, battenin

battenin

Battenin

BTS  [Similar Products]

CLN3_HUMAN

This gene encodes a protein that is involved in lysosomal function. Mutations in this, as well as other neuronal ceroid-lipofuscinosis (CLN) genes, cause neurodegenerative diseases commonly known as Batten disease or collectively known as neuronal ceroid lipofuscinoses (NCLs). Many alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2008]

CLN3: Involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes. Defects in CLN3 are the cause of neuronal ceroid lipofuscinosis type 3 (CLN3); also known as Batten disease. A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The hallmark of CLN3 is the ultrastructural pattern of lipopigment with a fingerprint profile, which can have 3 different appearances: pure within a lysosomal residual body; in conjunction with curvilinear or rectilinear profiles; and as a small component within large membrane-bound lysosomal vacuoles. The combination of fingerprint profiles within lysosomal vacuoles is a regular feature of blood lymphocytes from patients with CLN3. Belongs to the battenin family. 5 isoforms of the human protein are produced by alternative splicing.

Protein type: Apoptosis; Membrane protein, multi-pass; Membrane protein, integral; Autophagy; Chaperone; Vesicle; Mitochondrial

Chromosomal Location of Human Ortholog: 16p12.1

Cellular Component: autophagic vacuole; caveola; cytoplasm; early endosome; endoplasmic reticulum; Golgi apparatus; Golgi membrane; Golgi stack; integral to endoplasmic reticulum membrane; integral to membrane; late endosome; lipid raft; lysosomal membrane; lysosome; mitochondrion; neuron projection; nucleus; plasma membrane; synaptic vesicle; trans-Golgi network

Molecular Function: protein binding; unfolded protein binding

Biological Process: amino acid metabolic process; amyloid precursor protein catabolic process; arginine transport; associative learning; ceramide metabolic process; cytosolic calcium ion homeostasis; galactosylceramide metabolic process; globoside metabolic process; glucosylceramide metabolic process; ionotropic glutamate receptor signaling pathway; lysosomal lumen acidification; lysosome organization and biogenesis; macroautophagy; negative regulation of apoptosis; negative regulation of catalytic activity; negative regulation of macroautophagy; negative regulation of neuron apoptosis; negative regulation of proteolysis; neuromuscular process controlling balance; neurotransmitter metabolic process; protein catabolic process; protein processing; receptor-mediated endocytosis; regulation of action potential; sphingomyelin metabolic process; vacuolar transport; vesicle transport along microtubule

Disease: Ceroid Lipofuscinosis, Neuronal, 3

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