CLN6 sirna

CLN6 siRNA (Human)

Cat. Num. AAA8209147

• Gene Names: CLN6; nclf; CLN4A; HsT18960
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: CLN6; CLN6 siRNA (Human); Ceroid-lipofuscinosis neuronal protein 6; Protein CLN6; CLN6 sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: CLN6 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: 311

• Applicable Applications for CLN6 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 CLN6 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 CLN6 sirna

siRNA to inhibit CLN6 expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 8923532
• NCBI GeneID: 54982
• NCBI Accession #: NP_060352.1
• NCBI GenBank Nucleotide #: NM_017882.2
• UniProt Accession #: Q9NWW5; Q6IAB1; Q96SR0; A8K560; B4DDH6
• Molecular Weight: 39,484 Da
• NCBI Official Full Name: ceroid-lipofuscinosis neuronal protein 6
• NCBI Official Synonym Full Names: ceroid-lipofuscinosis, neuronal 6, late infantile, variant
• NCBI Official Symbol: CLN6
• NCBI Official Synonym Symbols: nclf; CLN4A; HsT18960
• NCBI Protein Information: ceroid-lipofuscinosis neuronal protein 6
• UniProt Protein Name: Ceroid-lipofuscinosis neuronal protein 6
• Protein Family: Ceroid-lipofuscinosis neuronal protein
• UniProt Gene Name: CLN6
• UniProt Synonym Gene Names: Protein CLN6
• UniProt Entry Name: CLN6_HUMAN

NCBI Description

This gene is one of eight which have been associated with neuronal ceroid lipofuscinoses (NCL). Also referred to as Batten disease, NCL comprises a class of autosomal recessive, neurodegenerative disorders affecting children. The genes responsible likely encode proteins involved in the degradation of post-translationally modified proteins in lysosomes. The primary defect in NCL disorders is thought to be associated with lysosomal storage function. [provided by RefSeq, Oct 2008]

Uniprot Description

CLN6: Defects in CLN6 are the cause of neuronal ceroid lipofuscinosis type 6 (CLN6). 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 lipopigment patterns observed most often in neuronal ceroid lipofuscinosis type 6 comprise mixed combinations of granular, curvilinear, and fingerprint profiles. Defects in CLN6 are the cause of neuronal ceroid lipofuscinosis type 4A (CLN4A). An adult-onset 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. CLN4A has no visual involvement and is characterized by progressive myoclonic epilepsy.

Protein type: Membrane protein, multi-pass; Membrane protein, integral

Chromosomal Location of Human Ortholog: 15q23

Cellular Component: nucleoplasm; endoplasmic reticulum membrane; nuclear membrane; intracellular membrane-bound organelle; membrane; endoplasmic reticulum lumen; endoplasmic reticulum; integral to membrane

Molecular Function: protein binding; protein homodimerization activity

Biological Process: cholesterol metabolic process; visual perception; glycosaminoglycan metabolic process; positive regulation of proteolysis; lysosomal lumen acidification; protein catabolic process; locomotion during locomotory behavior; ganglioside metabolic process; cellular macromolecule catabolic process

Disease: Ceroid Lipofuscinosis, Neuronal, 6; Ceroid Lipofuscinosis, Neuronal, 4a, Autosomal Recessive