背景：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.
仕様
サイズ：500ug
種由来：Human
発現宿主：HEK293 Cells
純度：＞97 ％ as determined by reducing SDS-PAGE.
エンドトキシン濃度：＜1.0 EU per μg of the protein as determined by the LAL method.
アクセッションNo.：NP_000478.2
アミノ酸配列：Met 1-Ala 507
遺伝子構造：A DNA sequence encoding the human Arylsulfatase A （NP_000478.2） （Met 1-Ala 507） was expressed with a C-terminal polyhistidine tag.
組成：Lyophilized from sterile 25mM Tris, 0.15mM NaCl, pH 7.5
保存条件：Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80℃. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at ＜ -20℃ for 3 months.
