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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
フォーム
buffered aqueous glycerol solution
交差性
human
強化検証
orthogonal RNAseq
Learn more about Antibody Enhanced Validation
テクニック
immunohistochemistry: 1:500-1:1000
免疫原配列
LLLGTGKSPRQSLFFYPSYPDEVRGVFAVRTGKYKAHFFTQGSAHSDTTADPACHASSSLTAHEPPLLYDLSKDPGENYNLLGGVAGATPEVLQALKQLQLLKAQLDAAVTFGPSQVARGEDPALQICCHPGCTPRPACCHCP
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 ... ARSA(410)
詳細
ARSA (arylsulfatase A) is a lysosomal sulfatase. This gene maps to human chromosome 22q13. This protein exists as a dimer and has a molecular weight of 107kDa. It is an acidic glycoprotein. It consists of 507 amino acid residues. Placental arylsulfatase A contains a putative 18 amino acid long signal peptide, preceding its N- terminal. ARSA is predicted to have three N-glycosylation sites.
免疫原
アリ-ルスルファタ-ゼA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
Anti-ARSA antibody is suitable for co-immunoprecipitation, co-localization and flow cytometry.
Anti-ARSA antibody produced in rabbit, a Prestige Antibody, 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. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
Anti-ARSA antibody produced in rabbit, a Prestige Antibody, 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. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
生物化学的／生理学的作用
ARSA (arylsulfatase A) requires post-translational modification to become catalytically active. This involves the oxidation of the -CH2SH group of a cysteine residue to aldehyde. Lack of this modification results in inactive enzyme, which causes the lysosomal storage disorder called multiple sulfatase deficiency. Complete or partial inactivation of this gene leads to Metachromatic leukodystrophy (MLD), which is a lysosomal storage disease. It is characterized by the accumulation of cerebroside sulfate in lysosomes. ARSA pseudo-deficiency is caused in individuals who are homozygous for the allele, which results in significant loss of ARSA activity. However, the activity is sufficient for normal cerebroside catabolism and results in clinically healthy phenotype.
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 APREST73374
物理的形状
PBS溶液 (pH 7.2, 40%グリセロ-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
HPA005554-25UL:
HPA005554-100UL:
最新バージョンのいずれかを選択してください：
Ruben J Boado et al.
Biotechnology and bioengineering, 110(5), 1456-1465 (2012-11-30)
Metachromatic leukodystrophy (MLD) is a lysosomal storage disorder of the brain caused by mutations in the gene encoding the lysosomal sulfatase, arylsulfatase A (ASA). It is not possible to treat the brain in MLD with recombinant ASA, because the enzyme
C DeLuca et al.
Proceedings of the National Academy of Sciences of the United States of America, 76(4), 1957-1961 (1979-04-01)
Genetics of human lysosomal arylsulfatases A and B (aryl-sulfate sulfohydrolase, EC 3.1.6.1), associated with childhood disease, has been studied with human-rodent somatic cell hybrids. Deficiency of arylsulfatase A (ARS(A)) in humans results in a progressive neurodegenerative disease, metachromatic leukodystrophy. Deficiency
V Gieselmann et al.
American journal of human genetics, 49(2), 407-413 (1991-08-01)
We identified a patient suffering from late infantile metachromatic leukodystrophy who genetically seemed to be homozygous for the mutations signifying the arylsulfatase A pseudodeficiency allele. Homozygosity for the pseudodeficiency allele is associated with low arylsulfatase A activity but does not
G Lukatela et al.
Biochemistry, 37(11), 3654-3664 (1998-04-02)
Human lysosomal arylsulfatase A (ASA) is a prototype member of the sulfatase family. These enzymes require the posttranslational oxidation of the -CH2SH group of a conserved cysteine to an aldehyde, yielding a formylglycine. Without this modification sulfatases are catalytically inactive
Agnieszka Ługowska et al.
PloS one, 6(6), e20218-e20218 (2011-06-23)
Metachromatic leukodystrophy (MLD) is a severe neurometabolic disease caused mainly by deficiency of arylsulfatase A encoded by the ARSA gene. Based on epidemiological surveys the incidence of MLD per 100,000 live births varied from 0.6 to 2.5. Our purpose was
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。.
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