Battenin; Batten disease protein; Protein CLN3; CLN3; BTS

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

Polyclonal

Rabbit Ig

Rabbit

This CLN3 antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 250-284 amino acids from the Central region of human CLN3.

Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

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.

Involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes.

Crown Antibodies; Cell Biology; Neuroscience

Flow Cytometry (FC/FACS), ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

FC~~1:25
IHC~~1:25
WB~~1:1000

Flow cytometric analysis of Hela cells using CLN3 Antibody (Center)(green) compared to an isotype control of rabbit IgG(blue). MBS9211565 was diluted at 1:25 dilution. An Alexa Fluor 488 goat anti-rabbit lgG at 1:400 dilution was used as the secondary antibody.

Immunohistochemical analysis of paraffin-embedded H. liver section using CLN3 Antibody (Center). MBS9211565 was diluted at 1:25 dilution. A undiluted biotinylated goat polyvalent antibody was used as the secondary, followed by DAB staining.

Western blot analysis of lysates from A431, KG-1, SH-SY5Y cell line, mouse kidney, mouse liver tissue (from left to right), using CLN3 Antibody (Center). MBS9211565 was diluted at 1:1000 at each lane. A goat anti-rabbit IgG H&L(HRP) at 1:10000 dilution was used as the secondary antibody. Lysates at 20ug per lane.

47623

ceroid-lipofuscinosis, neuronal 3

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

Chromosomal Location of Human Ortholog: 16p12.1

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

Molecular Function: protein binding; unfolded protein binding

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

Disease: Ceroid Lipofuscinosis, Neuronal, 3

Lerner T.J.,et al.Cell 82:949-957(1995).
Mitchison H.M.,et al.Genomics 40:346-350(1997).
LaFauci G.,et al.Submitted (JUL-1997) to the EMBL/GenBank/DDBJ databases.
LaFauci G.,et al.Submitted (JUL-1998) to the EMBL/GenBank/DDBJ databases.
Ota T.,et al.Nat. Genet. 36:40-45(2004).

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