Human palmitoyl-protein thioesterase 1 ELISA Kit | PPT1 elisa kit
Human Palmitoyl-protein thioesterase 1, PPT1 ELISA Kit
NCBI and Uniprot Product Information
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; EC 3.1.2.22; Hydrolase
Chromosomal Location of Human Ortholog: 1p32
Cellular Component: Golgi apparatus; extracellular space; synaptic vesicle; cell soma; membrane; axon; lysosome; 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; negative regulation of apoptosis; protein depalmitoylation
Disease: Ceroid Lipofuscinosis, Neuronal, 1
Research Articles on PPT1
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Product Notes
The Human PPT1 ppt1 (Catalog #AAA9317749) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9317749 ELISA Kit recognizes Human PPT1. It is sometimes possible for the material contained within the vial of "palmitoyl-protein thioesterase 1, 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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