Arylsulfatase A (ARSA) is synthesized as a 52KDa lysosomal enzyme. It is a member of the sulfatase family that is required for the lysosomal degradation of cerebroside-3-sulfate, a sphingolipid sulfate ester and a major constituent of the myelin sheet. Arylsulfatase A is activated by a required co- or posttranslational modification with the oxidation of cysteine to formylglycine. Metachromatic leukodystrophy (MLD) is a lysosomal storage disease in the central and peripheral nervous systems with severe and progressive neurological symptoms caused by the deficiency of Arylsulfatase A. Deficiency of this enzyme is also found in apparently healthy individuals, a condition for which the term pseudodeficiency is introduced. ARSA forms dimers after receiving three N-linked oligosaccharides in the endoplasmic reticulum, and then the dimers are transported to the Golgi where they receive mannose 6-phosphate recognition markers. And thus, ARSA is transported and delivered to dense lysosomes in a mannose 6-phosphate receptor-dependent manner. It has been shown that within the lysosomes, the ARSA dimers can oligomerize to an octamer in a pH-dependent manner. The ARSA deficiency leads to metachromatic leucodystrophy (MLD), a lysosomal storage disorder associated with severe and progressive demyelination in he central and peripheral nervous system. Additionally, the serum level of arylsulfatase A might be helpful in diagnosis of lung and central nervous system cancer.
Product Name:
Human Arylsulfatase A/ARSA Recombinant Protein (RPES4180)
Product Code:
RPES4180
Size:
20µg
Species:
Human
Expressed Host:
HEK293 Cells
Synonyms:
Arylsulfatase A, ASA, Cerebroside-Sulfatase, ARSA
Accession:
NP_000478.2
Sequence:
Met 1-Ala 507
Fusion tag:
C-His
Activity:
Measured by its ability to hydrolyze the substrate 4-Nitrocatechol Sulfate (PNCS). The specific activity is >50 pmoles/min/µg.
Endotoxin:
<1.0 EU per µg as determined by the LAL method.
Protein Construction:
A DNA sequence encoding the human Arylsulfatase A (NP_000478.2) (Met 1-Ala 507) was expressed with a C-terminal polyhistidine tag.
Purity:
> 97 % as determined by reducing SDS-PAGE.
Mol Mass:
53 kDa
AP Mol Mass:
53 kDa
Formulation:
Lyophilized from sterile 25mM Tris, 0.15mM NaCl, pH 7.5
Shipping:
This product is provided as lyophilized powder which is shipped with ice packs.
Stability and Storage:
Lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months.
