货号 | MAB2485-SP |
别名 | ARSA; arylsulfatase A; ASA; Cerebroside 3-sulfatase; Cerebroside-sulfatase; EC 3.1.6; EC 3.1.6.8; MLD |
反应种属 | Human |
应用 | Western Blot(1 µg/mL) Immunoprecipitation(25 µg/mL) |
目标/特异性 | Detects human Arylsulfatase A/ARSA in direct ELISAs and Western blots. In direct ELISAs and Western blots, 20‑50% cross‑reactivity with recombinant mouse ARSA is observed. |
使用方法 | Western Blot: 1 µg/mL Immunoprecipitation: 25 µg/mL |
来源 | Reconstitute at 0.5 mg/mL in sterile PBS. |
产品组分 |
供应商 | R&D Systems |
Entrez Gene IDs | 410 (Human); 11883 (Mouse) |
纯化方式 | Protein A or G purified from hybridoma culture supernatant |
免疫原 | Mouse myeloma cell line NS0-derived recombinant human Arylsulfatase A/ARSA Arg19-Ala507 Accession # AAH14210 |
生物活性 | Human |
标记 | Unconjugated |
溶解方法 | Reconstitute at 0.5 mg/mL in sterile PBS. |
背景 | 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 (1). 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 (2). The deduced amino acid sequence of human ARSA consists of a signal peptide (residues 1-18) and a mature chain (residues 19-507) (3). Human ARSA has a predicted molecular mass of approximately 53 kDa and an apparent molecular mass of approximately 63 kDa in SDS-PAGE. The amino acid sequence of human ARSA shares 92%, 89%, 87%, and 86% identity with porcine, canine, mouse, and rat ARSA. |
运输条件 | Blue Ice |
存放说明 | 4℃ |
参考文献 |
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