CLN8 sirna

CLN8 siRNA (Human)

Cat. Num. AAA8200212

• Gene Names: CLN8; EPMR; C8orf61
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: CLN8; CLN8 siRNA (Human); C8orf61; Protein CLN8; CLN8 sirna

• Host: Synthetic
• Reactivity: Human
• 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
• Sequence Length: 286

• Applicable Applications for CLN8 sirna: RNA Interference (RNAi)
• 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.

Related Product Information for CLN8 sirna

siRNA to inhibit CLN8 expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 31083053
• NCBI GeneID: 2055
• NCBI Accession #: NP_061764.2
• NCBI GenBank Nucleotide #: NM_018941.3
• UniProt Accession #: Q9UBY8; Q86U71; Q96I95
• Molecular Weight: 32,787 Da
• NCBI Official Full Name: protein CLN8
• NCBI Official Synonym Full Names: ceroid-lipofuscinosis, neuronal 8
• NCBI Official Symbol: CLN8
• NCBI Official Synonym Symbols: EPMR; C8orf61
• NCBI Protein Information: protein CLN8
• UniProt Protein Name: Protein CLN8
• Protein Family: Protein
• UniProt Gene Name: CLN8
• UniProt Synonym Gene Names: C8orf61
• UniProt Entry Name: CLN8_HUMAN

NCBI Description

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 Description

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; adult 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