Anti -PPT1, ID (PPT1, PPT, Palmitoyl-protein thioesterase 1, Palmitoyl-protein hydrolase 1)

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

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

Polyclonal

IgG

Rabbit

Human

Affinity Purified
Purified by Protein A affinity chromatography.

Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

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 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.

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.

Antibodies; Abs to Enzymes

ELISA (EL/EIA), Western Blot (WB)

Suitable for use in Western Blot, ELISA
Dilution: ELISA: 1:1,000
Western Blot: 1:100-500

34,193 Da[Similar Products]

palmitoyl-protein thioesterase 1

palmitoyl-protein thioesterase 1; palmitoyl-protein hydrolase 1; ceroid-palmitoyl-palmitoyl-protein thioesterase 1

Palmitoyl-protein thioesterase 1

PPT; PPT-1??[Similar Products]

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; membrane; cell soma; axon; lysosome; dendrite; extracellular region; nucleus; cytosol; 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; 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; negative regulation of cell growth; brain development; lipid raft organization and biogenesis; lipid catabolic process; negative regulation of apoptosis; protein depalmitoylation

Disease: Ceroid Lipofuscinosis, Neuronal, 1

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.