Full Product Name
CLN3 siRNA (Human)
Product Synonym Names
BTS; Battenin; Batten disease protein; Protein CLN3
Product Gene Name
CLN3 sirna
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q13286
Specificity
CLN3 siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
Purity/Purification
> 97%
Form/Format
Lyophilized powder
Quality Control
Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
Directions for Use
We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
Components
We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of human CLN3 gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
Preparation and Storage
Shipped at 4 degree C. Store at -20 degree C for one year.
Negative Control
siRNA Negative Control (Catalog# MBS8241404) is a non-targeting 21 nt siRNA recommended as a negative control for experiments using targeted siRNA transfection.
Other Notes
Small volumes of CLN3 sirna 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.
Related Product Information for
CLN3 sirna
siRNA to inhibit CLN3 expression using RNA interference
Applications Tested/Suitable for CLN3 sirna
RNA Interference (RNAi)
NCBI/Uniprot data below describe general gene information for CLN3. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000077.1
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NCBI GenBank Nucleotide #
NM_000086.2
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UniProt Primary Accession #
Q13286
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UniProt Secondary Accession #
O00668; O95089; Q549S9; Q9UP09; Q9UP11; Q9UP12; Q9UP13; Q9UP14; B2R7J1; B4DXL3[Other Products]
UniProt Related Accession #
Q13286[Other Products]
Molecular Weight
45,155 Da
NCBI Official Full Name
battenin isoform a
NCBI Official Synonym Full Names
ceroid-lipofuscinosis, neuronal 3
NCBI Official Symbol
CLN3 [Similar Products]
NCBI Official Synonym Symbols
BTS; JNCL
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NCBI Protein Information
battenin
UniProt Protein Name
Battenin
UniProt Synonym Protein Names
Batten disease protein; Protein CLN3
UniProt Gene Name
CLN3 [Similar Products]
UniProt Synonym Gene Names
BTS [Similar Products]
UniProt Entry Name
CLN3_HUMAN
NCBI Summary for CLN3
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 Comments for CLN3
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: Autophagy; Vesicle; Chaperone; Mitochondrial; Apoptosis; Membrane protein, integral; Membrane protein, multi-pass
Chromosomal Location of Human Ortholog: 16p12.1
Cellular Component: Golgi apparatus; neuron projection; Golgi stack; mitochondrion; lysosome; lysosomal membrane; endoplasmic reticulum; early endosome; integral to membrane; autophagic vacuole; caveola; trans-Golgi network; 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
1. CLN3 mutation is associated with neuronal ceroid lipofuscinosis.
Precautions
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Disclaimer
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