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安全信息

HPA005554

Sigma-Aldrich

Anti-ARSA antibody produced in rabbit

enhanced validation

affinity isolated antibody, buffered aqueous glycerol solution

别名:

Anti-ASA antibody produced in rabbit, Anti-Arylsulfatase A precursor antibody produced in rabbit, Anti-Cerebroside-sulfatase antibody produced in rabbit

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About This Item

UNSPSC代码:
12352203
人类蛋白质图谱编号:
NACRES:
NA.41

生物来源

rabbit

质量水平

偶联物

unconjugated

抗体形式

affinity isolated antibody

抗体产品类型

primary antibodies

克隆

polyclonal

表单

buffered aqueous glycerol solution

种属反应性

human

增强验证

orthogonal RNAseq
Learn more about Antibody Enhanced Validation

技术

immunohistochemistry: 1:500-1:1000

免疫原序列

LLLGTGKSPRQSLFFYPSYPDEVRGVFAVRTGKYKAHFFTQGSAHSDTTADPACHASSSLTAHEPPLLYDLSKDPGENYNLLGGVAGATPEVLQALKQLQLLKAQLDAAVTFGPSQVARGEDPALQICCHPGCTPRPACCHCP

UniProt登记号

运输

wet ice

储存温度

−20°C

靶向翻译后修饰

unmodified

基因信息

human ... ARSA(410)

一般描述

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

Arylsulfatase A precursor recombinant protein epitope signature tag (PrEST)

应用

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.

生化/生理作用

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.

特点和优势

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.

联系

Corresponding Antigen APREST73374

外形

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

法律信息

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

免责声明

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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储存分类代码

10 - Combustible liquids

WGK

WGK 1

闪点(°F)

Not applicable

闪点(°C)

Not applicable

个人防护装备

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)

法规信息

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分析证书(COA)

Lot/Batch Number

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C Stein et al.
The Journal of biological chemistry, 264(2), 1252-1259 (1989-01-15)
A full length cDNA for human arylsulfatase A was cloned and sequenced. The predicted amino acid sequence comprises 507 residues. A putative signal peptide of 18 residues is followed by the NH2-terminal sequence of placental arylsulfatase A. One of the
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
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
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
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

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