Key features and details
- Mouse polyclonal to ARSA/ASA
- Suitable for: WB, IHC-P
- Reacts with: Human
- Isotype: IgG
Product nameAnti-ARSA/ASA antibody
See all ARSA/ASA primary antibodies
DescriptionMouse polyclonal to ARSA/ASA
Tested applicationsSuitable for: WB, IHC-Pmore details
Species reactivityReacts with: Human
Full length protein corresponding to Human ARSA/ASA aa 1-507.
- Lysate from 293T cells transfected with ARSA/ASA.
Previously labelled as ARSA.
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We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.
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Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage bufferpH: 7.4
Constituent: 2.68% PBS
Concentration information loading...
PurityProtein A purified
Our Abpromise guarantee covers the use of ab67089 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use at an assay dependent concentration. Detects a band of approximately 70 kDa (predicted molecular weight: 54 kDa).|
|IHC-P||Use a concentration of 3 µg/ml.|
FunctionHydrolyzes cerebroside sulfate.
Involvement in diseaseDefects 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 similaritiesBelongs to the sulfatase family.
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).
- Information by UniProt
- arsA antibody
- ARSA_HUMAN antibody
- arylsulfatase A antibody
Immunohistochemistry of human lymph node staining ARSA/ASA using ab67089 at 3 μg/ml.
All lanes : Anti-ARSA/ASA antibody (ab67089)
Lane 1 : ARSA transfected 293T cells lysate
Lane 2 : non transfected 293T cells lysate
All lanes : Goat Anti-Mouse IgG (H&L)-HRP Conjugated at 1/2500 dilution
Predicted band size: 54 kDa
Observed band size: 70 kDa why is the actual band size different from the predicted?
ab67089 has not yet been referenced specifically in any publications.