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Western Blot (WB) (Western Blot analysis of PPT1 expression in transfected 293T cell line by PPT1 polyclonal antibody. Lane 1: PPT1 transfected lysate (34.2kD). Lane 2: Non-transfected lysate.)

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

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

Gene Names
PPT1; PPT; CLN1; INCL
Reactivity
Human
Applications
Western Blot
Purity
Purified by Protein A Affinity Chromatography.
Synonyms
PPT1; Polyclonal Antibody; PPT1 (Palmitoyl-protein Thioesterase 1; Palmitoyl-protein Hydrolase 1; PPT-1; PPT) (AP); anti-PPT1 antibody
Ordering
For Research Use Only!
Host
Rabbit
Reactivity
Human
Clonality
Polyclonal
Isotype
IgG
Specificity
Recognizes human PPT1.
Purity/Purification
Purified by Protein A Affinity Chromatography.
Form/Format
Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phosphatase (AP).
Applicable Applications for anti-PPT1 antibody
Western Blot (WB)
Application Notes
Applications are based on unconjugated antibody.
Immunogen
Full length human PPT1, aa1-306 (NP_000301.1).
Immunogen Sequence
MASPGCLWLLAVALLPWTCASRALQHLDPPAPLPLVIWHGMGDSCCNPLSMGAIKKMVEKKIPGIYVLSLEIGKTLMEDVENSFFLNVNSQVTTVCQALAKDPKLQQGYNAMGFSQGGQFLRAVAQRCPSPPMINLISVGGQHQGVFGLPRCPGESSHICDFIRKTLNAGAYSKVVQERLVQAEYWHDPIKEDVYRNHSIFLADINQERGINESYKKNLMALKKFVMVKFLNDSIVDPVDSEWFGFYRSGQAKETIPLQETSLYTQDRLGLKEMDNAGQLVFLATEGDHLQLSEEWFYAHIIPFLG
Conjugate
AP
Note
Preservative Free
Preparation and Storage
Store product at 4 degree C. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western Blot analysis of PPT1 expression in transfected 293T cell line by PPT1 polyclonal antibody. Lane 1: PPT1 transfected lysate (34.2kD). Lane 2: Non-transfected lysate.)

Western Blot (WB) (Western Blot analysis of PPT1 expression in transfected 293T cell line by PPT1 polyclonal antibody. Lane 1: PPT1 transfected lysate (34.2kD). Lane 2: Non-transfected lysate.)
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

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Accession #
Molecular Weight
23,094 Da
NCBI Official Full Name
palmitoyl-protein thioesterase 1 isoform 1
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
UniProt Protein Name
Palmitoyl-protein thioesterase 1
UniProt Gene Name
PPT1
UniProt Synonym Gene Names
PPT; PPT-1
UniProt Entry Name
PPT1_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.[provided by RefSeq, Dec 2008]

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

Chromosomal Location of Human Ortholog: 1p32

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

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

Biological Process: adult locomotory behavior; associative learning; brain development; cellular protein catabolic process; cofactor metabolic process; cofactor transport; grooming behavior; lipid catabolic process; lipid raft organization and biogenesis; lysosomal lumen acidification; negative regulation of apoptosis; negative regulation of cell growth; negative regulation of neuron apoptosis; nervous system development; neuron development; neurotransmitter secretion; pinocytosis; positive regulation of pinocytosis; positive regulation of receptor-mediated endocytosis; protein catabolic process; protein depalmitoylation; protein transport; receptor-mediated endocytosis; regulation of phospholipase A2 activity; regulation of synapse structure and activity; response to stimulus; sphingolipid catabolic process; synaptic transmission; visual perception

Disease: Ceroid Lipofuscinosis, Neuronal, 1

Research Articles on PPT1

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Product Notes

The PPT1 ppt1 (Catalog #AAA6390505) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The PPT1 (Palmitoyl-protein Thioesterase 1, Palmitoyl-protein Hydrolase 1, PPT-1, PPT) (AP) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's PPT1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Applications are based on unconjugated antibody. Researchers should empirically determine the suitability of the PPT1 ppt1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "PPT1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.

Precautions

All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.

Disclaimer

Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.

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