| 產(chǎn)品編號(hào) | bs-15543R |
| 英文名稱 | Iduronate 2 sulfatase Rabbit pAb |
| 中文名稱 | 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. |
| 研究領(lǐng)域 | 細(xì)胞生物 免疫學(xué) 細(xì)胞類型標(biāo)志物 |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Human (predicted: Mouse,Rat,Sheep,Cow,Dog) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 47 kDa |
| 檢測(cè)分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Iduronate 2 sulfatase: 101-200/550 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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. SWISS: P22304 Gene ID: 3423 Database links: Entrez Gene: 3423 Human Omim: 309900 Human SwissProt: P22304 Human Unigene: 460960 Human |
| 產(chǎn)品圖片 |
Sample:
Hela Cell(Human)Lysate at 30 ug
Primary: Anti- Iduronate 2 sulfatase (bs-15543R)at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 47kD
Observed band size: 62kD
Sample:
293T Cell (Human) Lysate at 30 ug
Primary: Anti- Iduronate 2 sulfatase (bs-15543R)at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 47kD
Observed band size: 62kD
Paraformaldehyde-fixed, paraffin embedded (Human kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Iduronate 2 sulfatase) Polyclonal Antibody, Unconjugated (bs-15543R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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