PPT1 sirna

PPT1 siRNA (Human)

Cat. Num. AAA8216766

• Gene Names: PPT1; PPT; CLN1; INCL
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: PPT1; PPT1 siRNA (Human); PPT; Palmitoyl-protein thioesterase 1; PPT-1; Palmitoyl-protein hydrolase 1; PPT1 sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: PPT1 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: 203

• Applicable Applications for PPT1 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 PPT1 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 PPT1 sirna

siRNA to inhibit PPT1 expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 4506031
• NCBI GeneID: 5538
• NCBI Accession #: NP_000301.1
• NCBI GenBank Nucleotide #: NM_000310.3
• UniProt Accession #: P50897; Q6FGQ4; B4DY24
• Molecular Weight: 23,094 Da
• NCBI Official Full Name: palmitoyl-protein thioesterase 1 isoform 1
• NCBI Official Synonym Full Names: palmitoyl-protein thioesterase 1
• NCBI Official Symbol: PPT1
• NCBI Official Synonym Symbols: PPT; CLN1; INCL
• NCBI Protein Information: palmitoyl-protein thioesterase 1
• UniProt Protein Name: Palmitoyl-protein thioesterase 1
• Protein Family: Palmitoyl-protein thioesterase
• UniProt Gene Name: PPT1
• UniProt Synonym Gene Names: PPT; PPT-1
• UniProt Entry Name: PPT1_HUMAN

NCBI Description

The protein encoded by this gene is a small glycoprotein involved in the catabolism of lipid-modified proteins during lysosomal degradation. The encoded enzyme removes thioester-linked fatty acyl groups such as palmitate from cysteine residues. Defects in this gene are a cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1, or INCL) and neuronal ceroid lipofuscinosis 4 (CLN4). Two transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Dec 2008]

Uniprot Description

PPT1: Removes thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation. Prefers acyl chain lengths of 14 to 18 carbons. Defects in PPT1 are the cause of neuronal ceroid lipofuscinosis type 1 (CLN1). A form of neuronal ceroid lipofuscinosis with variable age at onset. Infantile, late- infantile, juvenile, and adult onset have been reported. 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 pattern seen most often in CLN1 is referred to as granular osmiophilic deposits (GROD). Belongs to the palmitoyl-protein thioesterase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Lipid Metabolism - fatty acid elongation in mitochondria; Hydrolase; EC 3.1.2.22

Chromosomal Location of Human Ortholog: 1p32

Cellular Component: Golgi apparatus; extracellular space; synaptic vesicle; cell soma; membrane; lysosome; axon; dendrite; extracellular region; nucleus; cytosol; lipid raft

Molecular Function: palmitoyl-(protein) hydrolase activity; palmitoyl-CoA hydrolase activity

Biological Process: pinocytosis; adult locomotory behavior; neurotransmitter secretion; positive regulation of receptor-mediated endocytosis; protein transport; visual perception; protein catabolic process; cofactor transport; negative regulation of neuron apoptosis; associative learning; grooming behavior; nervous system development; receptor-mediated endocytosis; regulation of phospholipase A2 activity; response to stimulus; positive regulation of pinocytosis; sphingolipid catabolic process; cellular protein catabolic process; regulation of synapse structure and activity; lysosomal lumen acidification; neuron development; cofactor metabolic process; brain development; negative regulation of cell growth; lipid raft organization and biogenesis; lipid catabolic process; protein depalmitoylation; negative regulation of apoptosis

Disease: Ceroid Lipofuscinosis, Neuronal, 1