HPA005554
Anti-ARSA antibody produced in rabbit
affinity isolated antibody, buffered aqueous glycerol solution
Synonyme(s) :
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
Produits recommandés
Source biologique
rabbit
Niveau de qualité
Conjugué
unconjugated
Forme d'anticorps
affinity isolated antibody
Type de produit anticorps
primary antibodies
Clone
polyclonal
Forme
buffered aqueous glycerol solution
Espèces réactives
human
Validation améliorée
orthogonal RNAseq
Learn more about Antibody Enhanced Validation
Technique(s)
immunohistochemistry: 1:500-1:1000
Séquence immunogène
LLLGTGKSPRQSLFFYPSYPDEVRGVFAVRTGKYKAHFFTQGSAHSDTTADPACHASSSLTAHEPPLLYDLSKDPGENYNLLGGVAGATPEVLQALKQLQLLKAQLDAAVTFGPSQVARGEDPALQICCHPGCTPRPACCHCP
Numéro d'accès UniProt
Conditions d'expédition
wet ice
Température de stockage
−20°C
Modification post-traductionnelle de la cible
unmodified
Informations sur le gène
human ... ARSA(410)
Description générale
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.
Immunogène
Arylsulfatase A precursor recombinant protein epitope signature tag (PrEST)
Application
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.
Actions biochimiques/physiologiques
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.
Caractéristiques et avantages
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.
Liaison
Corresponding Antigen APREST73374
Forme physique
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Informations légales
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
Clause de non-responsabilité
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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Code de la classe de stockage
10 - Combustible liquids
Classe de danger pour l'eau (WGK)
WGK 1
Point d'éclair (°F)
Not applicable
Point d'éclair (°C)
Not applicable
Équipement de protection individuelle
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
Certificats d'analyse (COA)
Recherchez un Certificats d'analyse (COA) en saisissant le numéro de lot du produit. Les numéros de lot figurent sur l'étiquette du produit après les mots "Lot" ou "Batch".
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Retrouvez la documentation relative aux produits que vous avez récemment achetés dans la Bibliothèque de documents.
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
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