Rabbit anti-Human PPT1 Polyclonal Antibody | anti-PPT1 antibody

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

Cat. Num. AAA624117

• Gene Names: PPT1; PPT; CLN1; INCL
• Reactivity: Human
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: Affinity Purified; Purified by Protein G affinity chromatography.
• Synonyms: PPT1; Polyclonal Antibody; CT (Palmitoyl-protein Thioesterase 1; Palmitoyl-protein Hydrolase 1; PPT-1; PPT); Anti -PPT1; anti-PPT1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human PPT1.
• Purity/Purification: Affinity Purified; Purified by Protein G affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, 0.09% sodium azide.

• Applicable Applications for anti-PPT1 antibody: ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: Suitable for use in ELISA, Western Blot, and Immunohistochemistry.; Dilution: ELISA: 1:1,000; Western Blot: 1:100-1:500; Immunohistochemistry: 1:50-1:100
• Immunogen: Synthetic peptide selected from the C-terminal region of human PPT1 (KLH).
• Preparation and Storage: 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.

Western Blot (WB)

(Western blot analysis of PPT1 Antibody (C-term) in 293 cell line lysates (35ug/lane). PPT1 (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(Formalin-fixed and paraffin-embedded human lung carcinoma tissue reacted with PPT1 antibody (C-term) , which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.)

Western Blot (WB)

(Western blot analysis of PPT1 (arrow) using rabbit polyclonal PPT1 Antibody (A284) . 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected (Lane 2) with the PPT1 gene.)

Related Product Information for anti-PPT1 antibody

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.

Product Categories/Family for anti-PPT1 antibody

Antibodies; Abs to Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 49457059
• NCBI GeneID: 5538
• UniProt Accession #: Q6FGQ4; P50897; Q5T0S0; Q5T0S4
• Molecular Weight: 34,193 Da
• NCBI Official Full Name: PPT1
• NCBI Official Synonym Full Names: palmitoyl-protein thioesterase 1
• NCBI Official Symbol: PPT1
• NCBI Official Synonym Symbols: PPT; CLN1; INCL
• NCBI Protein Information: palmitoyl-protein thioesterase 1; PPT-1; OTTHUMP00000004836; OTTHUMP00000004837; OTTHUMP00000004838; OTTHUMP00000230752; OTTHUMP00000231000; palmitoyl-protein hydrolase 1; ceroid-palmitoyl-palmitoyl-protein thioesterase 1
• UniProt Protein Name: PPT1 protein
• Protein Family: Palmitoyl-protein thioesterase
• UniProt Gene Name: PPT1
• UniProt Entry Name: Q6FGQ4_HUMAN

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.

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

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; 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; 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