ARSA protein (Human) (ab116931)
Constituents: 0.3% Glutathione, 0.79% Tris HCl
Best use within three months from the date of receipt of this protein.
Our Abpromise guarantee covers the use of ab116931 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ELISA||ELISA: Use at an assay dependent concentration.|
|SDS-PAGE||SDS-PAGE: Use at an assay dependent concentration.|
|WB||WB: Use at an assay dependent concentration. Recombinant protein.|
- arsAARSA_HUMANarylsulfatase A
- Arylsulfatase A component CAs 2AS AAs2ASAAW212749C230037L18RikCerebroside-sulfatasemetachromatic leucodystrophyMGC125207MLDOTTHUMP00000196546OTTHUMP00000196548TISP73
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.
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).
ARSA protein (Human) images
12.5% SDS-PAGE stained with Coomassie Blue showing ab116931 at approximately 81.84 kDa.
References for ARSA protein (Human) (ab116931)
ab116931 has not yet been referenced specifically in any publications.