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

Polyclonal

Rabbit

Affinity purified

Liquid

100ug/100ul (lot specific)

This product is stable for several weeks at 4 degree C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20 degree C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.

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.

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]

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

ELISA titer: 1:20,000-1:80,000
Western blot analysis: 1:200-1:1,000
Immunohistochemistry: 1:100-1:500
Optimal dilutions/concentrations should be determined by the end user

23,094 Da

palmitoyl-protein thioesterase 1

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

Palmitoyl-protein thioesterase 1

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: 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; membrane; cell soma; lysosome; axon; 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; 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

(1) Kamate,M. and Hattiholi,V. Novel neuroimaging finding in palmitoyl protein thioesterase-1-related neuronal ceroid lipofuscinosis. Pediatr. Neurol. 46 (5), 325-328 (2012)

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