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Key Documents

HPA021546

Sigma-Aldrich

Anti-PPT1 antibody produced in rabbit

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

Sinônimo(s):

Anti-PPT-1, Anti-Palmitoyl-protein hydrolase 1, Anti-Palmitoyl-protein thioesterase 1

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About This Item

Código UNSPSC:
12352203
Número do Atlas de Proteínas Humanas:

fonte biológica

rabbit

Nível de qualidade

conjugado

unconjugated

forma do anticorpo

affinity isolated antibody

tipo de produto de anticorpo

primary antibodies

clone

polyclonal

linha de produto

Prestige Antibodies® Powered by Atlas Antibodies

forma

buffered aqueous glycerol solution

reatividade de espécies

human

técnica(s)

immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:200-1:500

sequência de imunogênio

KFVMVKFLNDSIVDPVDSEWFGFYRSGQAKETIPLQETSLYTQDRLGLKEMDNAGQLVFLATEGDHLQLSEEWFY

nº de adesão UniProt

Condições de expedição

wet ice

temperatura de armazenamento

−20°C

modificação pós-traducional do alvo

unmodified

Informações sobre genes

human ... PPT1(5538)

Descrição geral

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.

Imunogênio

Palmitoyl-protein thioesterase 1 Precursor recombinant protein epitope signature tag (PrEST)

Aplicação

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.

Ações bioquímicas/fisiológicas

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.

Características e benefícios

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:
  • 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.

Ligação

Corresponding Antigen APREST75160

forma física

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide

Informações legais

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

Exoneração de responsabilidade

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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Código de classe de armazenamento

10 - Combustible liquids

Classe de risco de água (WGK)

WGK 1

Ponto de fulgor (°F)

Not applicable

Ponto de fulgor (°C)

Not applicable


Certificados de análise (COA)

Busque Certificados de análise (COA) digitando o Número do Lote do produto. Os números de lote e remessa podem ser encontrados no rótulo de um produto após a palavra “Lot” ou “Batch”.

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Visite a Biblioteca de Documentos

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
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.
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
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
Enzo Scifo et al.
Data in brief, 4, 207-216 (2015-07-29)
Mutations in the CLN1 gene that encodes Palmitoyl protein thioesterase 1 (PPT1) or CLN1, cause Infantile NCL (INCL, MIM#256730). PPT1 removes long fatty acid chains such as palmitate from modified cysteine residues of proteins. The data shown here result from

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