Anti -PPT1 (Palmitoyl-protein Thioesterase 1, Palmitoyl-protein Hydrolase 1, PPT-1, PPT)

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 1; NC_000001.10 (40538382..40563142, complement). Location: 1p32

Polyclonal

IgG

Mouse

Recognizes human PPT1.

Affinity Purified
Purified by Protein A affinity chromatography.

Supplied as a liquid in PBS, pH 7.2.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Small volumes of anti-PPT1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Palmitoyl-protein thioesterase-1 (PPT1) is a lysosomal hydrolase that removes long-chain fatty acyl groups from modified cysteine residues in proteins. Mutations in PPT1 have been found to cause the infantile form of neuronal ceroid lipofuscinosis (INCL), and an animal model has been developed.1 The deduced PPT2 protein contains 302 amino acids, including a 27aa leader peptide, a sequence motif characteristic of many thioesterases and lipases, and 5 potential N-linked glycosylation sites.2 PPT2 shares 18% amino acid identity with PPT1. Northern blot analysis detected a predominant 2.0-kb PPT2 transcript in the human tissues examined, with the highest expression in skeletal muscle; variable amounts of 2.8- and 7.0-kb transcripts were also observed. Recombinant PPT2, like PPT1, possesses thioesterase activity and localizes to the lysosome. Since PPT2 could not substitute for PPT1 in correcting the metabolic defect in INCL cells and was unable to remove palmitate groups from palmitoylated proteins that are routinely used as substrates for PPT1it has been postulated that PPT2 possesses a different substrate specificity than PPT1.

Antibodies; Abs to Proteins

Western Blot (WB), Immunohistochemistry (IHC)

Suitable for use in Western Blot and Immunohistochemistry.
Dilution: Immunohistochemistry (Formalin fixed paraffin embedded): 3ug/ml

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PPT1_HUMAN

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]

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

Chromosomal Location of Human Ortholog: 1p32

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

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

Biological Process: pinocytosis; ***** locomotory behavior; neurotransmitter secretion; positive regulation of receptor-mediated endocytosis; protein transport; visual perception; protein catabolic process; negative regulation of neuron apoptosis; cofactor transport; associative learning; grooming behavior; receptor-mediated endocytosis; nervous system development; 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; cofactor metabolic process; neuron development; 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

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