Cat#: | THP-0282 |
Product Name: | Arylsulfatase A (ARSA) |
Description: | Recombinant human ARSA (Arg19-Ala507) corresponds to the mature chain and has sulfatase activity described above. This protein is fused 10-His tag at the C-terminal. |
Introduction: | As a member of the sulfatase family, ARSA is required for the lysosomal degradation of cerebroside-3-sulfate, a sphingolipid sulfate ester and a major constituent of the myelin sheet. The ARSA deficiency results in metachromatic leukodystrophy (MLD), a lysosomal storage disease in the central and peripheral nervous systems with severe and progressive neurological symptoms. The deduced amino acid sequence of human ARSA consists of a signal peptide (residues 1-18) and a mature chain (residues 19-507). |
Species: | Human |
Source: | Mouse myeloma cell line |
Molecular Weight: | Predicted Molecular Mass: 53 kDa; SDS-PAGE: 63 kDa, reducing conditions. |
Synonyms: | ARSA; Arylsulfatase A; ASA; Cerebroside 3-sulfatase; Cerebroside-sulfatase; EC 3.1.6; EC 3.1.6.8; MLD; |
Biological Activity: | Measured by its ability to hydrolyze the substrate 4-Nitrocatechol Sulfate (PNCS). The specific activity is >25 pmol/min/µg, as measured under the described conditions. |
Purity: | >95%, by SDS-PAGE under reducing conditions and visualized by silver stain |
Formulation: | Supplied as a 0.2 μm filtered solution in Tris and NaCl. |
Storage: | Use a manual defrost freezer and avoid repeated freeze-thaw cycles. 6 months from date of receipt, -20 to -70 centigrade as supplied. 3 months, -20 to -70 centigrade under sterile conditions after opening. |
Endotoxin Level: | <1.0 EU per 1 μg of the protein by the LAL method. |
Applications: | The ARSA deficiency results in metachromatic leukodystrophy (MLD), a lysosomal storage disease in the central and peripheral nervous systems with severe and progressive neurological symptoms. |
Examples of Clinical Use: | Improving metachromatic leukodystrophy (MLD). |
Affected organisms: | Humans |
For research use only. Not intended for any clinical use. No products from Creative BioMart may be resold, modified for resale or used to manufacture commercial products without prior written approval from Creative BioMart.
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