HPA021546
抗PPT1 ウサギ宿主抗体
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
別名:
Anti-PPT-1, Anti-Palmitoyl-protein hydrolase 1, Anti-Palmitoyl-protein thioesterase 1
ログイン組織・契約価格を表示する
すべての画像(6)
About This Item
結合体:
unconjugated
application:
IHC
クローン:
polyclonal
化学種の反応性:
human
citations:
9
テクニック:
immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:200-1:500
immunohistochemistry: 1:200-1:500
おすすめの製品
由来生物
rabbit
品質水準
結合体
unconjugated
抗体製品の状態
affinity isolated antibody
抗体製品タイプ
primary antibodies
クローン
polyclonal
製品種目
Prestige Antibodies® Powered by Atlas Antibodies
フォーム
buffered aqueous glycerol solution
化学種の反応性
human
テクニック
immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:200-1:500
免疫原配列
KFVMVKFLNDSIVDPVDSEWFGFYRSGQAKETIPLQETSLYTQDRLGLKEMDNAGQLVFLATEGDHLQLSEEWFY
UniProtアクセッション番号
輸送温度
wet ice
保管温度
−20°C
ターゲットの翻訳後修飾
unmodified
遺伝子情報
human ... PPT1(5538)
詳細
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
パルミトイルプロテインチオエステラーゼ1前駆体のPrEST(protein epitope signature tag)抗原リコンビナントタンパク質。
アプリケーション
Prestige抗体®は、Human Proteome Resource(HPR)プロジェクト(www.proteinatlas.org)によって開発・実証されたAtlas抗体です。抗体はすべて、数百の正常組織・疾病組織に対する免疫組織染色試験を行っています。これらの染色画像はHuman Protein Atlas(HPA)サイトで[Image Gallery]リンクをクリックするとご覧いただけます。さらに、ほとんどのPrestige抗体はプロテインアレイおよびウェスタンブロッティングの試験を行っています。試験のプロトコールおよびPrestige抗体、HPAに関する情報はsigma.com/prestigeをご覧ください。
生物化学的／生理学的作用
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.
特徴および利点
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.
関連事項
Corresponding Antigen APREST75160
物理的形状
PBS溶液(pH 7.2, 40%グリセロールおよび0.02%アジ化ナトリウム含有)。
法的情報
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
免責事項
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.
適切な製品が見つかりませんか。
製品選択ツール.をお試しください
保管分類コード
10 - Combustible liquids
WGK
WGK 1
引火点(°F)
Not applicable
引火点(℃)
Not applicable
適用法令
試験研究用途を考慮した関連法令を主に挙げております。化学物質以外については、一部の情報のみ提供しています。 製品を安全かつ合法的に使用することは、使用者の義務です。最新情報により修正される場合があります。WEBの反映には時間を要することがあるため、適宜SDSをご参照ください。
Jan Code
HPA021546-25UL:
HPA021546-100UL:
最新バージョンのいずれかを選択してください：
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
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
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
ライフサイエンス、有機合成、材料科学、クロマトグラフィー、分析など、あらゆる分野の研究に経験のあるメンバーがおります。.
製品に関するお問い合わせはこちら（テクニカルサービス）