HPA005468
Anti-ASAH1 antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
別名:
Anti-AC, Anti-Acid ceramidase precursor, Anti-Acylsphingosine deacylase, Anti-N-acylsphingosine amidohydrolase, Anti-PHP32, Anti-Putative 32 kDa heart protein
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About This Item
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由来生物
rabbit
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
形状
buffered aqueous glycerol solution
化学種の反応性
human
強化検証
orthogonal RNAseq
Learn more about Antibody Enhanced Validation
テクニック
immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:500-1:1000
免疫原配列
ENSTSYEEAKNLLTKTKILAPAYFILGGNQSGEGCVITRDRKESLDVYELDAKQGRWYVVQTNYDRWKHPFFLDDRRTPAKMCLNRTSQENISFETMYDVLSTKPVLNKLTVYTTLIDVTKGQF
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 ... ASAH1(427)
詳細
N-acylsphingosine amidohydrolase (ASAH1) gene is located on human chromosome 8p22-21.2. It is a 26.5kb long gene, which has 14 exons and 13 introns. This gene codes for a 55kDa protein with two subunits- 13kDa α and 40kDa β subunits. It is a glycoprotein and the two subunits are produced by auto-proteolytic cleavage. This protein is highly expressed in heart, lung, kidney and placenta and is expressed to a lesser extent in brain, liver, pancreas and skeletal muscle. ASAH1 is localized to lysosomes and human fibroblasts and macrophages secrete it extracellularly.
免疫原
酸性セラミダーゼ前駆体の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
N-acylsphingosine amidohydrolase (ASAH1) converts ceramide to fatty acid and sphingosine. ASAH1 deficiency leads to Farber disease (FD) or Farber lipogranulomatosis, which is a lysosomal storage disease. It is characterized by pulmonary insufficiency, painful swelling of joints and tendons, and a shortened life-span, because of the accumulation of ceramide in tissues. It regulates adrenocortical steroidogenesis by maintaining the intracellular balance of ceramide, sphingosine and sphingosine-1-phosphate. These molecules in turn act as second messengers in protein kinase A/cAMP-dependent pathway mediated steroidogenesis. Expression of ASAH1 is found to be elevated in multiple tumor types, especially prostate cancer. Studies suggest that ASAH1 is a potential therapeutic target in chemoresistant and advanced prostate cancer types.
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 APREST70303
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.
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保管分類コード
10 - Combustible liquids
WGK
WGK 1
引火点(°F)
Not applicable
引火点(℃)
Not applicable
個人用保護具 (PPE)
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
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
HPA005468-100UL:
試験成績書（COA）
製品のロット番号・バッチ番号を入力して、試験成績書（COA） を検索できます。ロット番号・バッチ番号は、製品ラベルに「Lot」または「Batch」に続いて記載されています。
Justine Leclerc et al.
Oncogene, 38(8), 1282-1295 (2018-09-27)
Phenotypic plasticity and subsequent generation of intratumoral heterogeneity underly key traits in malignant melanoma such as drug resistance and metastasis. Melanoma plasticity promotes a switch between proliferative and invasive phenotypes characterized by different transcriptional programs of which MITF is a
Luz Camacho et al.
Journal of lipid research, 54(5), 1207-1220 (2013-02-21)
Acid ceramidase (AC) catalyzes the hydrolysis of ceramide into sphingosine, in turn a substrate of sphingosine kinases that catalyze its conversion into the mitogenic sphingosine-1-phosphate. AC is expressed at high levels in several tumor types and has been proposed as
Z Zhang et al.
Molecular genetics and metabolism, 70(4), 301-309 (2000-09-20)
Farber disease is an autosomal recessive disorder caused by lysosomal acid ceramidase (AC) deficiency. It commonly manifests during the first few months after birth with a unique triad of painful and progressive deformed joints, subcutaneous nodules, and progressive hoarseness. In
C M Li et al.
Genomics, 62(2), 223-231 (1999-12-28)
Acid ceramidase (AC) is the lysosomal enzyme that degrades ceramide into sphingosine and fatty acid. A deficiency in human AC activity leads to the lysosomal storage disorder, Farber disease (FD). The human AC gene (HGMW-approved symbol ASAH) was cloned and
Natasha Lucki et al.
Biochimica et biophysica acta, 1791(8), 706-713 (2009-03-21)
Acid ceramidase (encoded by ASAH1) is a lipid hydrolase that catalyzes the conversion of ceramide (cer) into sphingosine (SPH) and a free fatty acid. Adrenocortical steroidogenesis is regulated by the trophic peptide hormone adrenocorticotropin (ACTH), which induces the expression of
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