Western Blot (WB)
(Western Blot analysis of CLN3 expression in transfected 293T cell line by CLN3 monoclonal antibody.|Lane 1: CLN3 transfected lysate (47.623kD).|Lane 2: Non-transfected lysate.)
Mouse anti-Human CLN3 Monoclonal Antibody | anti-CLN3 antibody
CLN3 (Battenin, Batten Disease Protein, Protein CLN3, BTS)
Purified by Protein A affinity chromatography.
Purified by Protein A affinity chromatography.
Western Blot (WB)
(Western Blot analysis of CLN3 expression in transfected 293T cell line by CLN3 monoclonal antibody.|Lane 1: CLN3 transfected lysate (47.623kD).|Lane 2: Non-transfected lysate.)
Western Blot (WB)
(Western Blot analysis of CLN3 expression in transfected 293T cell line by CLN3 monoclonal antibody.|Lane 1: CLN3 transfected lysate (47.623kD).|Lane 2: Non-transfected lysate.)
Testing Data
(Detection limit for recombinant GST tagged CLN3 is 0.3ng/ml as a capture antibody.)
Testing Data
(Detection limit for recombinant GST tagged CLN3 is 0.3ng/ml as a capture antibody.)
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
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: Mitochondrial; Membrane protein, integral; Vesicle; Chaperone; Apoptosis; Membrane protein, multi-pass; Autophagy
Chromosomal Location of Human Ortholog: 16p12.1
Cellular Component: Golgi apparatus; Golgi stack; neuron projection; mitochondrion; endoplasmic reticulum; lysosomal membrane; lysosome; early endosome; integral to membrane; autophagic vacuole; trans-Golgi network; caveola; lipid raft; Golgi membrane; synaptic vesicle; cytoplasm; late endosome; 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; negative regulation of proteolysis; amino acid metabolic process; regulation of action potential; receptor-mediated endocytosis; cytosolic calcium ion homeostasis; vacuolar transport; arginine transport; globoside metabolic process; galactosylceramide metabolic process; lysosome organization and biogenesis; ionotropic glutamate receptor signaling pathway; negative regulation of catalytic activity; lysosomal lumen acidification; protein processing; ceramide metabolic process; negative regulation of apoptosis
Disease: Ceroid Lipofuscinosis, Neuronal, 3
Research Articles on CLN3
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Product Notes
The CLN3 cln3 (Catalog #AAA641166) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The CLN3 (Battenin, Batten Disease Protein, Protein CLN3, BTS) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's CLN3 can be used in a range of immunoassay formats including, but not limited to, ELISA (EL/EIA), Western Blot (WB). Suitable for use in ELISA and Western Blot. Researchers should empirically determine the suitability of the CLN3 cln3 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. The amino acid sequence is listed below: MGGCAGSRRR FSDSEGEETV PEPRLPLLDH QGAHWKNAVG FWLLGLCNNF SYVVMLSAAH DILSHKRTSG NQSHVDPGPT PIPHNSSSRF DCNSVSTAAV LLADILPTLV IKLLAPLGLH LLPYSPRVLV SGICAAGSFV LVAFSHSVGT SLCGVVFASI SSGLGEVTFL SLTAFYPRAV ISWWSSGTGG AGLLGALSYL GLTQAGLSPQ QTLLSMLGIP ALLLASYFLL LTSPEAQDPG GEEEAESAAR QPLIRTEAPE SKPGSSSSLS LRERWTVFKG LLWYIVPLVV VYFAEYFINQ GLFELLFFWN TSLSHAQQYR WYQMLYQAGV FASRSSLRCC RIRFTWALAL LQCLNLVFLL ADVWFGFLPS IYLVFLIILY EGLLGGAAYV NTFHNIALET SDEHREFAMA ATCISDTLGI SLSGLLALPL HDFLCQLS. It is sometimes possible for the material contained within the vial of "CLN3, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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