Mouse Palmitoyl-Protein Thioesterase 1 (PPT1) ELISA Kit | PPT1 elisa kit
Mouse Palmitoyl-Protein Thioesterase 1 (PPT1) ELISA Kit
* Allow all reagents to warm to room temperature for at least 30 minutes before opening.
* Pre-rinse the pipet tip with reagent, use fresh pipet tips for each sample, standard and reagent to avoid contamination.
* Unused wells must be kept desiccated at 4 degree C in the sealed bag provided.
* Mix Thoroughly is very important for the result. It is recommended using low frequency oscillator or slight hand shaking every 10 minutes.
* It is recommended that all samples and standards be assayed in duplicate or triplicate.
Background: PPT1 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. The cDNA predicted a 306-amino acid polypeptide that contains a 25-amino acid signal peptide, 3 N-linked glycosylation sites, and consensus motifs characteristic of thioesterases. Northern blot analysis revealed ubiquitous expression of a single 2.5-kb mRNA, with highest expression in lung, brain, and heart.
NCBI and Uniprot Product Information
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
Cellular Component: Golgi apparatus; extracellular space; synaptic vesicle; neuron projection; membrane; cell soma; axon; lysosome; dendrite; extracellular region; synapse; nucleus; lipid raft
Molecular Function: hydrolase activity; palmitoyl-(protein) hydrolase activity; palmitoyl-CoA hydrolase activity
Biological Process: grooming behavior; pinocytosis; nervous system development; receptor-mediated endocytosis; regulation of phospholipase A2 activity; adult locomotory behavior; neurotransmitter secretion; positive regulation of pinocytosis; positive regulation of receptor-mediated endocytosis; protein transport; visual perception; lysosome organization and biogenesis; cellular protein catabolic process; lysosomal lumen acidification; cofactor metabolic process; cofactor transport; negative regulation of neuron apoptosis; brain development; negative regulation of cell growth; lipid raft organization and biogenesis; lipid catabolic process; associative learning; cellular macromolecule catabolic process; negative regulation of apoptosis; protein depalmitoylation
Research Articles on PPT1
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Product Notes
The Mouse PPT1 ppt1 (Catalog #AAA9717306) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9717306 ELISA Kit recognizes Mouse PPT1. It is sometimes possible for the material contained within the vial of "Palmitoyl-Protein Thioesterase 1 (PPT1), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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