Recombinant Human ARSA/ASA protein is a Human Fragment protein, in the 19 to 507 aa range, expressed in HEK 293, with >95% purity, < 1 EU/µg endotoxin level and suitable for SDS-PAGE, HPLC.
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| Application | Reactivity | Dilution info | Notes |
|---|---|---|---|
Application SDS-PAGE | Reactivity Reacts | Dilution info - | Notes - |
Application HPLC | Reactivity Reacts | Dilution info - | Notes - |
Arylsulfatase A, ASA, Cerebroside-sulfatase, ARSA
Recombinant Human ARSA/ASA protein is a Human Fragment protein, in the 19 to 507 aa range, expressed in HEK 293, with >95% purity, < 1 EU/µg endotoxin level and suitable for SDS-PAGE, HPLC.
pH: 7.5
Constituents: 0.88% Sodium chloride, 0.32% Tris HCl
Hydrolyzes cerebroside sulfate.
Belongs to the sulfatase family.
The 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).
Previously labelled as ARSA.
The ARSA protein also known as arylsulfatase A or ASA protein functions mechanically as a lysosomal enzyme responsible for the cleavage of cerebroside sulfate into cerebroside and sulfate. It belongs to a family of enzymes important for the hydrolysis of sulfate esters and has a molecular mass of about 62 kDa. This enzyme is expressed mainly in the liver and kidney but also in the brain where it plays important roles in normal functioning.
ARSA is essential in the breakdown of sulfatides which are components of myelin sheaths in the central nervous system. It functions as part of a lysosomal complex involving cooperators like saposin B which facilitates its interaction with sulfatide substrates. By breaking down sulfatides ARSA helps in cellular membrane maintenance and recycling contributing to neurological function and stability.
ARSA is mainly involved in the sphingolipid metabolism pathway. This pathway manages the degradation of sphingolipids important for maintaining neuron and cell membrane integrity. ARSA also interacts with proteins such as galactosylceramidase as both play roles in lipid recycling and are essential for maintaining cell health within the nervous system.
ARSA's deficiency is a well-known factor in metachromatic leukodystrophy (MLD) a genetic disorder leading to progressive leukodystrophy. This condition results from sulfatide accumulation in the nervous system caused by insufficient ARSA activity. The disease involves other proteins such as saposin B which when dysfunctional exacerbate ARSA-related pathologies and symptoms. Another disorder related to defective ARSA function includes chronic kidney disease where compromised sulfatide metabolism further effects cellular processes.
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