Anti-ARSA/ASA antibody (ab154300)
Key features and details
- Rabbit polyclonal to ARSA/ASA
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
Get better batch-to-batch reproducibility with a recombinant antibody
- Research with confidence – consistent and reproducible results with every batch
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Overview
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Product name
Anti-ARSA/ASA antibody
See all ARSA/ASA primary antibodies -
Description
Rabbit polyclonal to ARSA/ASA -
Host species
Rabbit -
Tested applications
Suitable for: WBmore details -
Species reactivity
Reacts with: Human
Predicted to work with: Mouse, Rat, Cow, Dog -
Immunogen
Recombinant fragment corresponding to Human ARSA/ASA aa 169-405 (internal sequence).
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Positive control
- A431; H1299; HeLa and HepG2 cell lysates.
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General notes
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle. -
Storage buffer
pH: 7.00
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 1.21% Tris, 0.75% Glycine, 10% Glycerol (glycerin, glycerine) -
Concentration information loading...
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Purity
Immunogen affinity purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
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Positive Controls
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Recombinant Protein
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab154300 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application | Abreviews | Notes |
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WB |
1/500 - 1/3000. Predicted molecular weight: 54 kDa.
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Notes |
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WB
1/500 - 1/3000. Predicted molecular weight: 54 kDa. |
Target
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Function
Hydrolyzes cerebroside sulfate. -
Involvement in disease
Defects in ARSA are a cause of leukodystrophy metachromatic (MLD) [MIM:250100]. MLD is a disease due to a lysosomal storage defect. It is characterized by intralysosomal storage of cerebroside-3-sulfate in neural and non-neural tissues, with a diffuse loss of myelin in the central nervous system. Progressive demyelination causes a variety of neurological symptoms, including gait disturbances, ataxias, optical atrophy, dementia, seizures, and spastic tetraparesis. Three forms of the disease can be distinguished according to the age at onset: late-infantile, juvenile and adult.
Arylsulfatase A activity is defective in multiple sulfatase deficiency (MSD) [MIM:272200]. MSD is a disorder characterized by decreased activity of all known sulfatases. MSD is due to defects in SUMF1 resulting in the lack of post-translational modification of a highly conserved cysteine into 3-oxoalanine. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay. -
Sequence similarities
Belongs to the sulfatase family. -
Post-translational
modificationsThe conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity. This post-translational modification is severely defective in multiple sulfatase deficiency (MSD). -
Cellular localization
Lysosome. - Information by UniProt
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Database links
- Entrez Gene: 505514 Cow
- Entrez Gene: 474457 Dog
- Entrez Gene: 410 Human
- Entrez Gene: 11883 Mouse
- Entrez Gene: 315222 Rat
- Omim: 607574 Human
- SwissProt: Q08DD1 Cow
- SwissProt: P15289 Human
see all -
Alternative names
- arsA antibody
- ARSA_HUMAN antibody
- arylsulfatase A antibody
see all
Images
Datasheets and documents
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SDS download
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Datasheet download
References (0)
ab154300 has not yet been referenced specifically in any publications.