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Anti-GNS antibody produced in mouse
| 产品编号： | 3365371 |
| 规格： | purified immunoglobulin, buffered aqueous solution |
| 包装规格： | 50 μG |
| 产品类别： | 进口试剂 |
| 品牌： | Sigma-Aldrich |
| 优惠价： | 立即咨询 |
产品别名
Anti-GNS antibody produced in mouse
G6S
MGC21274
基本信息
| NACRES | NA.41 |
| General description【一般描述】 | The product of this gene is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome. (provided by RefSeq) |
| Immunogen【免疫原】 | GNS (NP_002067.1, 1 a.a. ~ 552 a.a) full-length human protein. Sequence MRLLPLAPGRLRRGSPRHLPSCSPALLLLVLGGCLGVFGVAAGTRRPNVVLLLTDDQDEVLGGMTPLKKTKALIGEMGMTFSSAYVPSALCCPSRASILTGKYPHNHHVVNNTLEGNCSSKSWQKIQEPNTFPAILRSMCGYQTFFAGKYLNEYGAPDAGGLEHVPLGWSYWYALEKNSKYYNYTLSINGKARKHGENYSVDYLTDVLANVSLDFLDYKSNFEPFFMMIATPAPHSPWTAAPQYQKAFQNVFAPRNKNFNIHGTNKHWLIRQAKTPMTNSSIQFLDNAFRKRWQTLLSVDDLVEKLVKRLEFTGELNNTYIFYTSDNGYHTGQFSLPIDKRQLYEFDIKVPLLVRGPGIKPNQTSKMLVANIDLGPTILDIAGYDLNKTQMDGMSLLPILRGASNLTWRSDVLVEYQGEGRNVTDPTCPSLSPGVSQCFPDCVCEDAYNNTYACVRTMSALWNLQYCEFDDQEVFVEVYNLTADPDQITNIAKTIDPELLGKMNYRLMMLQSCSGPTCRTPGVFDPGYRFDPRLMFSNRGSVRTRRFSKHLL |
| Physical form【外形】 | Solution in phosphate buffered saline, pH 7.4 |
产品性质
| Quality Level【质量水平】 | 100 |
| biological source【生物来源】 | mouse |
| conjugate【偶联物】 | unconjugated |
| antibody form【抗体形式】 | purified immunoglobulin |
| antibody product type | primary antibodies |
| clone【克隆】 | polyclonal |
| form【形式】 | buffered aqueous solution |
| mol wt【分子量】 | antigen ~62.1 kDa |
| species reactivity | human |
| technique(s) | western blot: 1 μg/mL |
| NCBI accession no.【NCBI登记号】 | NM_002076.2 |
| UniProt accession no.【UniProt登记号】 | P15586 |
| shipped in【运输】 | dry ice |
| storage temp.【储存温度】 | −20℃ |
| Gene Information | human ... GNS(2799) |
安全信息
| Storage Class Code【储存分类代码】 | 13 - Non Combustible Solids |
| WGK | WGK 1 |
