Palmitoyl-protein thioesterase 1; PPT-1; Palmitoyl-protein hydrolase 1; PPT1; PPT

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

Monoclonal

IgG1

1117CT11.2.1.4

Mouse

This PPT1 antibody is generated from a mouse immunized with a full-length recombinant protein from human PPT1.

Purified Mouse Monoclonal Antibody (Mab)

Purified monoclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein G column, followed by dialysis against PBS.

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

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.

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.

Crown Antibodies; Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA), Flow Cytometry (FC/FACS), Immunohistochemistry (IHC)

WB~~1:1000
FC~~1:25
IHC~~1:25

All lanes : Anti-PPT1 Antibody (C-term) at 1:2000 dilution
Lane 1: Hela whole cell lysates
Lane 2: HepG2 whole cell lysates

Lysates/proteins at 20 ug per lane.

Secondary
Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution

Predicted band size : 34 kDa

Blocking/Dilution buffer: 5% NFDM/TBST.

Flow cytometric analysis of HepG2 cells using PPT1 Antibody (C-term)(green) compared to an isotype control of mouse IgG1(blue). MBS9200355 was diluted at 1:25 dilution. An Alexa Fluor 488 goat anti-mouse lgG at 1:400 dilution was used as the secondary antibody.

Immunohistochemical analysis of paraffin-embedded H. cerebellum section using PPT1 Antibody (C-term). MBS9200355 was diluted at 1:25 dilution. A peroxidase-conjugated goat anti-rabbit IgG at 1:400 dilution was used as the secondary antibody, followed by DAB staining.

Immunohistochemical analysis of paraffin-embedded M. cerebellum section using PPT1 Antibody (C-term). MBS9200355 was diluted at 1:25 dilution. A peroxidase-conjugated goat anti-rabbit IgG at 1:400 dilution was used as the secondary antibody, followed by DAB staining.

Immunohistochemical analysis of paraffin-embedded R. cerebellum section using PPT1 Antibody (C-term). MBS9200355 was diluted at 1:25 dilution. A peroxidase-conjugated goat anti-rabbit IgG at 1:400 dilution was used as the secondary antibody, followed by DAB staining.

Western blot analysis of lysate from HepG2 cell line using PPT1 Antibody. MBS9200355 was diluted at 1:1000 at each lane. A goat anti-mouse IgG H&L(HRP) at 1:3000 dilution was used as the secondary antibody. Lysate at 35ug per lane.

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palmitoyl-protein thioesterase 1

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: 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; membrane; cell soma; lysosome; axon; 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; 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; negative regulation of cell growth; brain development; lipid raft organization and biogenesis; lipid catabolic process; protein depalmitoylation; negative regulation of apoptosis

Disease: Ceroid Lipofuscinosis, Neuronal, 1

Vesa J.,et al.Nature 376:584-587(1995).
Crews C.M.,et al.Proc. Natl. Acad. Sci. U.S.A. 93:4316-4319(1996).
Schriner J.E.,et al.Genomics 34:317-322(1996).
Ota T.,et al.Nat. Genet. 36:40-45(2004).
Ebert L.,et al.Submitted (JUN-2004) to the EMBL/GenBank/DDBJ databases.

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