Full Product Name
CLN8 siRNA (Human)
Product Synonym Names
C8orf61; Protein CLN8
Product Gene Name
CLN8 sirna
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q9UBY8
Specificity
CLN8 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 CLN8 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 CLN8 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
CLN8 sirna
siRNA to inhibit CLN8 expression using RNA interference
Applications Tested/Suitable for CLN8 sirna
RNA Interference (RNAi)
NCBI/Uniprot data below describe general gene information for CLN8. It may not necessarily be applicable to this product.
NCBI Accession #
NP_061764.2
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NCBI GenBank Nucleotide #
NM_018941.3
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UniProt Primary Accession #
Q9UBY8
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UniProt Secondary Accession #
Q86U71; Q96I95[Other Products]
UniProt Related Accession #
Q9UBY8[Other Products]
Molecular Weight
32,787 Da
NCBI Official Full Name
protein CLN8
NCBI Official Synonym Full Names
ceroid-lipofuscinosis, neuronal 8
NCBI Official Symbol
CLN8??[Similar Products]
NCBI Official Synonym Symbols
EPMR; C8orf61
??[Similar Products]
NCBI Protein Information
protein CLN8
UniProt Protein Name
Protein CLN8
UniProt Gene Name
CLN8??[Similar Products]
UniProt Synonym Gene Names
C8orf61??[Similar Products]
UniProt Entry Name
CLN8_HUMAN
NCBI Summary for CLN8
This gene encodes a transmembrane protein belonging to a family of proteins containing TLC domains, which are postulated to function in lipid synthesis, transport, or sensing. The protein localizes to the endoplasmic reticulum (ER), and may recycle between the ER and ER-Golgi intermediate compartment. Mutations in this gene are associated with progressive epilepsy with mental retardation (EMPR), which is a subtype of neuronal ceroid lipofuscinoses (NCL). Patients with mutations in this gene have altered levels of sphingolipid and phospholipids in the brain. [provided by RefSeq, Jul 2008]
UniProt Comments for CLN8
CLN8: Could play a role in cell proliferation during neuronal differentiation and in protection against cell death. Defects in CLN8 are the cause of neuronal ceroid lipofuscinosis type 8 (CLN8). A form of neuronal ceroid lipofuscinosis with onset in childhood. 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 lipopigment patterns observed most often in neuronal ceroid lipofuscinosis type 8 comprise mixed combinations of granular, curvilinear, and fingerprint profiles. Defects in CLN8 are the cause of neuronal ceroid lipofuscinosis type 8 Northern epilepsy variant (CLN8NE). A form of neuronal ceroid lipofuscinosis clinically characterized by epilepsy that presents between 5 and 10 years of age with frequent tonic-clonic seizures followed by progressive mental retardation. Visual loss is not a prominent feature. Intracellular accumulation of autofluorescent material results in curvilinear and granular profiles on ultrastructural analysis.
Protein type: Membrane protein, multi-pass; Membrane protein, integral; Cell development/differentiation; Apoptosis; Endoplasmic reticulum
Chromosomal Location of Human Ortholog: 8p23
Cellular Component: endoplasmic reticulum membrane; ER-Golgi intermediate compartment membrane; mitochondrion; endoplasmic reticulum; integral to membrane; ER-Golgi intermediate compartment
Biological Process: negative regulation of proteolysis; nervous system development; cholesterol metabolic process; mitochondrial membrane organization and biogenesis; photoreceptor cell maintenance; lipid biosynthetic process; glutamate uptake during transmission of nerve impulse; social behavior; lipid transport; ***** walking behavior; age-dependent response to oxidative stress; neuromuscular process controlling posture; negative regulation of transferase activity; neurofilament cytoskeleton organization and biogenesis; visual perception; lysosome organization and biogenesis; somatic motor neuron differentiation; retina development in camera-type eye; cellular protein catabolic process; phospholipid metabolic process; ceramide biosynthetic process; protein catabolic process; neuromuscular process controlling balance; musculoskeletal movement; ceramide metabolic process; regulation of cell size; associative learning; negative regulation of apoptosis
Disease: Ceroid Lipofuscinosis, Neuronal, 8; Ceroid Lipofuscinosis, Neuronal, 8, Northern Epilepsy Variant
Research Articles on CLN8
1. This study highlights a close interaction between CLN5/CLN8 proteins, and their role in sphingolipid metabolism. Our findings suggest that CLN5p/CLN8p most likely are positive modulators of CerS1 and/or CerS2.
Precautions
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Disclaimer
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