推荐产品
生物源
rabbit
共軛
unconjugated
抗體表格
affinity isolated antibody
抗體產品種類
primary antibodies
無性繁殖
polyclonal
產品線
Prestige Antibodies® Powered by Atlas Antibodies
形狀
buffered aqueous glycerol solution
物種活性
human
技術
immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:200-1:500
免疫原序列
KFVMVKFLNDSIVDPVDSEWFGFYRSGQAKETIPLQETSLYTQDRLGLKEMDNAGQLVFLATEGDHLQLSEEWFY
UniProt登錄號
運輸包裝
wet ice
儲存溫度
−20°C
目標翻譯後修改
unmodified
基因資訊
human ... PPT1(5538)
一般說明
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.
免疫原
Palmitoyl-protein thioesterase 1 Precursor recombinant protein epitope signature tag (PrEST)
應用
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.
生化／生理作用
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.
特點和優勢
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.
聯結
Corresponding Antigen APREST75160
外觀
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
法律資訊
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
免責聲明
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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儲存類別代碼
10 - Combustible liquids
水污染物質分類（WGK）
WGK 1
閃點（°F）
Not applicable
閃點（°C）
Not applicable
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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