Recombinant Human Arylsulfatase A/ARSA Protein (His Tag)

Specifications

Catalog number

PKSH031600-01

Size / count

50 µg

Dry ice

No

Hazardous

No

Discontinued

No

Background

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.

Synonyms

ARSA; ASA; Arylsulfatase A; Cerebroside-Sulfatase

Accession Number

NP_000478.2

Expression System

HEK293 Cells

Tag

C-His

Sequence

Met 1-Ala 507

Applications

Enzyme

Endotoxin

< 1.0 EU per μg of the protein as determined by the LAL method.

Purity

> 97 % as determined by reducing SDS-PAGE.

Bioactivity

Measured by its ability to hydrolyze the substrate 4-Nitrocatechol Sulfate (PNCS) . The specific activity is > 50 pmoles/min/μg.

Reconstitution

Please refer to the printed manual for detailed information.

Shipping Conditions

This product is provided as lyophilized powder which is shipped with ice packs.

Storage Conditions

Generally, 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.

Calculated Molecular Weight

53.0 kDa

Observed Molecular Weight

53 kDa

Species

Human