| 產(chǎn)品編號 | bs-15543R-BF647 |
| 英文名稱(chēng) | Rabbit Anti-Iduronate 2 sulfatase/BF647 Conjugated antibody |
| 中文名稱(chēng) | BF647標記的艾杜糖-2-硫酸酯酶抗體 |
| 別 名 | Alpha L iduronate sulfate sulfatase; Alpha-L-iduronate sulfate sulfatase; AW214631; Ids; IDS_HUMAN; Iduronate 2 sulfatase 14 kDa chain; Iduronate 2 sulfatase 42 kDa chain; Iduronate 2 sulfatase; Iduronate 2-sulfatase 14 kDa chain; Iduronate sulfatase; Idursulfase; MPS2; RP23-29M4.1; SIDS. |
| 規格價(jià)格 | 100ul/2980元 購買(mǎi) 大包裝/詢(xún)價(jià) |
| 說(shuō) 明 書(shū) | 100ul |
| 研究領(lǐng)域 | 細胞生物 免疫學(xué) 細胞類(lèi)型標志物 |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 交叉反應 | Human, (predicted: Mouse, Rat, Dog, Cow, Sheep, ) |
| 產(chǎn)品應用 | ICC=1:50-200 IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 47kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Iduronate 2 sulfatase |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 產(chǎn)品介紹 |
background: The protein encoded by this gene belongs to the sulfatase family, is localized to the lysosome, and is involved in lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this gene are associated with the X-linked lysosomal storage disease, mucopolysaccharidosis type II, also known as Hunter syndrome. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Aug 2013] Function: Required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Subunit: Liver iduronate 2-sulfatase is composed of two major forms (A and B) which contain both a 42 kDa and a 14 kDa polypeptides. Subcellular Location: Lysosome. Tissue Specificity: Liver, kidney, lung, and placenta. Post-translational modifications: The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity (By similarity). DISEASE: Mucopolysaccharidosis 2 (MPS2) [MIM:309900]: An X-linked lysosomal storage disease characterized by intracellular accumulation of heparan sulfate and dermatan sulfate and their excretion in urine. Most children with MPS2 have a severe form with early somatic abnormalities including skeletal deformities, hepatosplenomegaly, and progressive cardiopulmonary deterioration. A prominent feature is neurological damage that presents as developmental delay and hyperactivity but progresses to mental retardation and dementia. They die before 15 years of age, usually as a result of obstructive airway disease or cardiac failure. In contrast, those with a mild form of MPS2 may survive into adulthood, with attenuated somatic complications and often without mental retardation. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the sulfatase family. Database links: Entrez Gene: 3423 Human Omim: 309900 Human SwissProt: P22304 Human Unigene: 460960 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
