HPA021546
Anti-PPT1 antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
Synonym(s):
Anti-PPT-1, Anti-Palmitoyl-protein hydrolase 1, Anti-Palmitoyl-protein thioesterase 1
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All Photos(5)
About This Item
Recommended Products
biological source
rabbit
Quality Level
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
product line
Prestige Antibodies® Powered by Atlas Antibodies
form
buffered aqueous glycerol solution
species reactivity
human
technique(s)
immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:200-1:500
immunogen sequence
KFVMVKFLNDSIVDPVDSEWFGFYRSGQAKETIPLQETSLYTQDRLGLKEMDNAGQLVFLATEGDHLQLSEEWFY
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... PPT1(5538)
General description
The gene coding for the depalmitoylation enzyme, palmitoyl-protein thioesterase (PPT1) is mapped to human chromosome 1p32. Ser115–His289–Asp233 amino acids form the catalytic triad in the active site of the protein. Even though PPT1 is expressed in a variety of tissues, inadequacy of PPT1 protein affect nerve cells only in the cerebral, cerebellar cortex and retina, all other cell types remain comparatively unaffected.
Immunogen
Palmitoyl-protein thioesterase 1 Precursor recombinant protein epitope signature tag (PrEST)
Application
Anti-PPT1 antibody produced in rabbit has been used for immunofluorescence and western blotting studies. All Prestige Antibodies®Powered by Atlas Antibodies is developed and validated by the Human Protein Atlas (HPA) project . Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
Biochem/physiol Actions
Palmitoyl-protein thioesterase-1 (PPT1) enzyme catalyzes the depalmitoylation reaction involving the removal of long-chain fatty acids from modified cysteine residues in proteins. Mutation of this gene leads to infantile neuronal ceroid lipofuscinosis (INCL), which is an autosomal recessive disorder, characterized by brain atrophy, myoclonic jerk and visual abnormalities.
Features and Benefits
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
Linkage
Corresponding Antigen APREST75160
Physical form
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Legal Information
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Storage Class Code
10 - Combustible liquids
WGK
WGK 1
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Certificates of Analysis (COA)
Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.
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David C Cantu et al.
Protein science : a publication of the Protein Society, 19(7), 1281-1295 (2010-05-28)
Thioesterases (TEs) are classified into EC 3.1.2.1 through EC 3.1.2.27 based on their activities on different substrates, with many remaining unclassified (EC 3.1.2.-). Analysis of primary and tertiary structures of known TEs casts a new light on this enzyme group.
Yo Niida et al.
Brain & development, 38(7), 674-677 (2016-02-06)
Infantile neuronal ceroid lipofuscinosis (INCL) is an autosomal recessive disorder starting in infancy as early as 12-month-old, caused by PPT1 (palmitoyl-protein thioesterase 1) mutations, and characterized by progressive psychomotor deterioration, brain atrophy, myoclonic jerk and visual impairment. INCL can be
Laura Ahtiainen et al.
The Journal of comparative neurology, 455(3), 368-377 (2002-12-17)
Palmitoyl protein thioesterase 1 (PPT1) is a depalmitoylating enzyme whose deficiency leads to infantile neuronal ceroid lipofuscinosis. The disease is characterized by early loss of vision and massive neuronal death. Although PPT1 is expressed in many tissues, a deficiency of
J J Bellizzi et al.
Proceedings of the National Academy of Sciences of the United States of America, 97(9), 4573-4578 (2000-04-26)
Mutations in palmitoyl-protein thioesterase 1 (PPT1), a lysosomal enzyme that removes fatty acyl groups from cysteine residues in modified proteins, cause the fatal inherited neurodegenerative disorder infantile neuronal ceroid lipofuscinosis. The accumulation of undigested substrates leads to the formation of
Michal Segal-Salto et al.
PloS one, 11(1), e0146466-e0146466 (2016-01-06)
Mutations in the depalmitoylating enzyme gene, PPT1, cause the infantile form of Neuronal Ceroid Lipofuscinosis (NCL), an early onset neurodegenerative disease. During recent years there have been different therapeutic attempts including enzyme replacement. Here we show that PPT1 is palmitoylated
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